Lymphoma & Plasma Cell Disorders

Background: Cat scratch disease is a benign, self-limiting but rare condition that causes significant lymphadenopathy. There is less than 1 case per 100,000 cases per year, mostly in children. Even rarer are cases presenting in association with malignancies, usually non-Hodgkin lymphoma. Our review of the literature revealed only 1 case of cat scratch disease as-sociated with Hodgkin lymphoma. We describe here a case of a 43-year-old white man who presented with a clinical picture of recurrent Hodgkin lymphoma but with negative serology for Bartonella antibodies, responding to a short course of antibiotic treatment.

Methods: This 43-year-old patient initially presented with stage IA Hodgkin lymphoma in January 2011. He had a me-diastinal mass, which was resected and followed by 4 cycles of ABVD (doxorubicin, bleomycin, vincristine, dexameth-asone) chemotherapy. In July 2011, he went into complete remission after 4 cycles of chemotherapy and remained so until October 2014, when he noted increasing left-sided in-guinal adenopathy accompanied by a constant stabbing pain. He had no other symptoms. On positron emission tomogra-phy/computed tomography (PET/CT) scanning he had sev-eral hypermetabolic splenic lesions and enlarged tonsillar, retroperitoneal, pelvic, axillary, and inguinal lymph nodes. His complete blood count and chemistries were normal. His sedimentation rate was also normal. Bilateral bone marrow biopsies were negative for Hodgkin disease recurrence. Bi-opsy of one of his inguinal nodes was positive for necrotiz-ing granulomas and stained positive for CD2, CD3, and CD5 with areas of high Ki-67 proliferation. Hodgkin disease, how-ever, was not confirmed. He mentioned a family history of cat scratch disease a decade ago when his 2 young children were diagnosed with cat scratch disease and treated with oral antibiotics, resulting in complete resolution of symptoms. Se-rologic testing for Bartonella antibodies was ordered for him but came back negative. An infectious disease consult was re-quested, and he was treated empirically with a 5-day course of azithromycin.

Results: After 2 months, he was seen in the clinic and found to have no palpable inguinal adenopathy. A repeat PET/CT scan at 3 months showed resolution of all areas of lymphadenop-athy with the exception of the left inguinal node basin. At 5 months, all areas of hypermetabolic adenopathy had resolved. A repeat serology for Bartonella antibodies remained negative.

Conclusions: Cat scratch disease is extremely rare but can occur in a patient with known hematologic malignancies. In this case, it was mistaken as a recurrent case of relapsed Hodgkin disease but fortunately resolved quickly. The pre-sentation can be acute; patients can have negative serologies for Bartonellosis, yet achievement of a complete clinical and radiographic response with a short course of oral antibiotics was successful.

Background: Cat scratch disease is a benign, self-limiting but rare condition that causes significant lymphadenopathy. There is less than 1 case per 100,000 cases per year, mostly in children. Even rarer are cases presenting in association with malignancies, usually non-Hodgkin lymphoma. Our review of the literature revealed only 1 case of cat scratch disease as-sociated with Hodgkin lymphoma. We describe here a case of a 43-year-old white man who presented with a clinical picture of recurrent Hodgkin lymphoma but with negative serology for Bartonella antibodies, responding to a short course of antibiotic treatment.

Methods: This 43-year-old patient initially presented with stage IA Hodgkin lymphoma in January 2011. He had a me-diastinal mass, which was resected and followed by 4 cycles of ABVD (doxorubicin, bleomycin, vincristine, dexameth-asone) chemotherapy. In July 2011, he went into complete remission after 4 cycles of chemotherapy and remained so until October 2014, when he noted increasing left-sided in-guinal adenopathy accompanied by a constant stabbing pain. He had no other symptoms. On positron emission tomogra-phy/computed tomography (PET/CT) scanning he had sev-eral hypermetabolic splenic lesions and enlarged tonsillar, retroperitoneal, pelvic, axillary, and inguinal lymph nodes. His complete blood count and chemistries were normal. His sedimentation rate was also normal. Bilateral bone marrow biopsies were negative for Hodgkin disease recurrence. Bi-opsy of one of his inguinal nodes was positive for necrotiz-ing granulomas and stained positive for CD2, CD3, and CD5 with areas of high Ki-67 proliferation. Hodgkin disease, how-ever, was not confirmed. He mentioned a family history of cat scratch disease a decade ago when his 2 young children were diagnosed with cat scratch disease and treated with oral antibiotics, resulting in complete resolution of symptoms. Se-rologic testing for Bartonella antibodies was ordered for him but came back negative. An infectious disease consult was re-quested, and he was treated empirically with a 5-day course of azithromycin.

Results: After 2 months, he was seen in the clinic and found to have no palpable inguinal adenopathy. A repeat PET/CT scan at 3 months showed resolution of all areas of lymphadenop-athy with the exception of the left inguinal node basin. At 5 months, all areas of hypermetabolic adenopathy had resolved. A repeat serology for Bartonella antibodies remained negative.

Conclusions: Cat scratch disease is extremely rare but can occur in a patient with known hematologic malignancies. In this case, it was mistaken as a recurrent case of relapsed Hodgkin disease but fortunately resolved quickly. The pre-sentation can be acute; patients can have negative serologies for Bartonellosis, yet achievement of a complete clinical and radiographic response with a short course of oral antibiotics was successful.

Background: Cat scratch disease is a benign, self-limiting but rare condition that causes significant lymphadenopathy. There is less than 1 case per 100,000 cases per year, mostly in children. Even rarer are cases presenting in association with malignancies, usually non-Hodgkin lymphoma. Our review of the literature revealed only 1 case of cat scratch disease as-sociated with Hodgkin lymphoma. We describe here a case of a 43-year-old white man who presented with a clinical picture of recurrent Hodgkin lymphoma but with negative serology for Bartonella antibodies, responding to a short course of antibiotic treatment.

Methods: This 43-year-old patient initially presented with stage IA Hodgkin lymphoma in January 2011. He had a me-diastinal mass, which was resected and followed by 4 cycles of ABVD (doxorubicin, bleomycin, vincristine, dexameth-asone) chemotherapy. In July 2011, he went into complete remission after 4 cycles of chemotherapy and remained so until October 2014, when he noted increasing left-sided in-guinal adenopathy accompanied by a constant stabbing pain. He had no other symptoms. On positron emission tomogra-phy/computed tomography (PET/CT) scanning he had sev-eral hypermetabolic splenic lesions and enlarged tonsillar, retroperitoneal, pelvic, axillary, and inguinal lymph nodes. His complete blood count and chemistries were normal. His sedimentation rate was also normal. Bilateral bone marrow biopsies were negative for Hodgkin disease recurrence. Bi-opsy of one of his inguinal nodes was positive for necrotiz-ing granulomas and stained positive for CD2, CD3, and CD5 with areas of high Ki-67 proliferation. Hodgkin disease, how-ever, was not confirmed. He mentioned a family history of cat scratch disease a decade ago when his 2 young children were diagnosed with cat scratch disease and treated with oral antibiotics, resulting in complete resolution of symptoms. Se-rologic testing for Bartonella antibodies was ordered for him but came back negative. An infectious disease consult was re-quested, and he was treated empirically with a 5-day course of azithromycin.

Results: After 2 months, he was seen in the clinic and found to have no palpable inguinal adenopathy. A repeat PET/CT scan at 3 months showed resolution of all areas of lymphadenop-athy with the exception of the left inguinal node basin. At 5 months, all areas of hypermetabolic adenopathy had resolved. A repeat serology for Bartonella antibodies remained negative.

Conclusions: Cat scratch disease is extremely rare but can occur in a patient with known hematologic malignancies. In this case, it was mistaken as a recurrent case of relapsed Hodgkin disease but fortunately resolved quickly. The pre-sentation can be acute; patients can have negative serologies for Bartonellosis, yet achievement of a complete clinical and radiographic response with a short course of oral antibiotics was successful.

Background: The purpose of this study was to identify an association between dioxin (Agent Orange) exposure and development of cutaneous T-cell lymphoma (CTCL) in U.S. veterans. Cutaneous lymphomas are cancers characterized by malignant transformation of lymphocytes, or white blood cells, which affect the skin. Definitive causative factors of CTCL have not been identified, although there have been reports linking CTCL to dioxin exposure. However, these studies have been small and unsubstantiated. During military service, many soldiers were exposed to dioxin during the Korean and Vietnam conflicts. Significantly, despite CTCL being a very rare lymphoma, there are 37 veterans diagnosed with CTCL in the VA system, which is probably an underestimation, considering the difficulty in diagnosis.

Methods: We performed a retrospective chart analysis of 37 veterans with cutaneous T-cell lymphoma, identified via the VA Informatics and Computing Infrastructure (VINCI) database, and cross-referenced these individuals with the Agent Orange Registry to characterize extent and duration of dioxin exposure. These patients were compared with Vietnam-era patients with hepatitis C.

Conclusions: The association of CTCL with dioxin exposure allowed for the identification of veterans with dioxin exposure and history of chronic dermatitis such that they can be monitored prospectively for earlier diagnosis of CTCL; early detection of CTCL translates to early treatment and potential prevention of disease progression.

Background: The purpose of this study was to identify an association between dioxin (Agent Orange) exposure and development of cutaneous T-cell lymphoma (CTCL) in U.S. veterans. Cutaneous lymphomas are cancers characterized by malignant transformation of lymphocytes, or white blood cells, which affect the skin. Definitive causative factors of CTCL have not been identified, although there have been reports linking CTCL to dioxin exposure. However, these studies have been small and unsubstantiated. During military service, many soldiers were exposed to dioxin during the Korean and Vietnam conflicts. Significantly, despite CTCL being a very rare lymphoma, there are 37 veterans diagnosed with CTCL in the VA system, which is probably an underestimation, considering the difficulty in diagnosis.

Methods: We performed a retrospective chart analysis of 37 veterans with cutaneous T-cell lymphoma, identified via the VA Informatics and Computing Infrastructure (VINCI) database, and cross-referenced these individuals with the Agent Orange Registry to characterize extent and duration of dioxin exposure. These patients were compared with Vietnam-era patients with hepatitis C.

Conclusions: The association of CTCL with dioxin exposure allowed for the identification of veterans with dioxin exposure and history of chronic dermatitis such that they can be monitored prospectively for earlier diagnosis of CTCL; early detection of CTCL translates to early treatment and potential prevention of disease progression.

Background: The purpose of this study was to identify an association between dioxin (Agent Orange) exposure and development of cutaneous T-cell lymphoma (CTCL) in U.S. veterans. Cutaneous lymphomas are cancers characterized by malignant transformation of lymphocytes, or white blood cells, which affect the skin. Definitive causative factors of CTCL have not been identified, although there have been reports linking CTCL to dioxin exposure. However, these studies have been small and unsubstantiated. During military service, many soldiers were exposed to dioxin during the Korean and Vietnam conflicts. Significantly, despite CTCL being a very rare lymphoma, there are 37 veterans diagnosed with CTCL in the VA system, which is probably an underestimation, considering the difficulty in diagnosis.

Methods: We performed a retrospective chart analysis of 37 veterans with cutaneous T-cell lymphoma, identified via the VA Informatics and Computing Infrastructure (VINCI) database, and cross-referenced these individuals with the Agent Orange Registry to characterize extent and duration of dioxin exposure. These patients were compared with Vietnam-era patients with hepatitis C.

Conclusions: The association of CTCL with dioxin exposure allowed for the identification of veterans with dioxin exposure and history of chronic dermatitis such that they can be monitored prospectively for earlier diagnosis of CTCL; early detection of CTCL translates to early treatment and potential prevention of disease progression.

Purpose: Today, patients with relapsed low-grade or follicular B-cell non-Hodgkin lymphoma have more treatment options available to them than ever. These therapies include infused chemotherapy regimens, radioimmunotherapy, and oral agents. There is limited information available about the impact of available treatment sequences on clinical outcomes and costs.

Methods: A budget impact model was developed to represent the current treatment pathways for relapsed low-grade or follicular B-cell non-Hodgkin lymphoma. First-line treatment regimens were selected based on the Category 1 recommendations from the National Comprehensive Cancer Network guidelines for follicular lymphoma. Those included were the most commonly used: BR, RCHOP, and RCVP. For purposes of this model, the second-line regimens included Y90 ibritumomab (Y90), rituximab monotherapy with 4 weekly doses, and idelalisib. Treatment outcomes were based on the published literature that summarized the overall response rate, median duration of response (DOR), and toxicity. Total costs per treatment regimen were based on medical care, drugs administered, and associated adverse effects. The 2015 Medicare fee schedule rates were used to estimate the medical care costs. Total DOR and costs were determined for each treatment regimen.

Results: The treatment sequence, BR followed by Y90 at the time of relapse, had the longest predicted DOR (7.2 years). The associated treatment sequence costs were $157,712 for BR followed by Y90, compared with $224,628 and $145,047 for BR followed by idelalisib and BR followed by rituximab monotherapy, respectively, with a predicted DOR of 6.7 years. The predicted DOR for treatment sequences starting with RCVP and RCHOP and followed by Y90 was about 3 years less than BR followed by Y90 at a cost increase of $11,102 and $37,866, respectively. The use of idelalisib after RCVP or RCHOP had a predicted DOR of 3.6 years, respectively, with associated costs that were greater than BR followed by Y90.

Conclusions: The use of Y90 ibritumomab after BR demonstrated the optimal patient outcomes at one of the lowest cost profiles.

Purpose: Today, patients with relapsed low-grade or follicular B-cell non-Hodgkin lymphoma have more treatment options available to them than ever. These therapies include infused chemotherapy regimens, radioimmunotherapy, and oral agents. There is limited information available about the impact of available treatment sequences on clinical outcomes and costs.

Methods: A budget impact model was developed to represent the current treatment pathways for relapsed low-grade or follicular B-cell non-Hodgkin lymphoma. First-line treatment regimens were selected based on the Category 1 recommendations from the National Comprehensive Cancer Network guidelines for follicular lymphoma. Those included were the most commonly used: BR, RCHOP, and RCVP. For purposes of this model, the second-line regimens included Y90 ibritumomab (Y90), rituximab monotherapy with 4 weekly doses, and idelalisib. Treatment outcomes were based on the published literature that summarized the overall response rate, median duration of response (DOR), and toxicity. Total costs per treatment regimen were based on medical care, drugs administered, and associated adverse effects. The 2015 Medicare fee schedule rates were used to estimate the medical care costs. Total DOR and costs were determined for each treatment regimen.

Results: The treatment sequence, BR followed by Y90 at the time of relapse, had the longest predicted DOR (7.2 years). The associated treatment sequence costs were $157,712 for BR followed by Y90, compared with $224,628 and $145,047 for BR followed by idelalisib and BR followed by rituximab monotherapy, respectively, with a predicted DOR of 6.7 years. The predicted DOR for treatment sequences starting with RCVP and RCHOP and followed by Y90 was about 3 years less than BR followed by Y90 at a cost increase of $11,102 and $37,866, respectively. The use of idelalisib after RCVP or RCHOP had a predicted DOR of 3.6 years, respectively, with associated costs that were greater than BR followed by Y90.

Conclusions: The use of Y90 ibritumomab after BR demonstrated the optimal patient outcomes at one of the lowest cost profiles.

Purpose: Today, patients with relapsed low-grade or follicular B-cell non-Hodgkin lymphoma have more treatment options available to them than ever. These therapies include infused chemotherapy regimens, radioimmunotherapy, and oral agents. There is limited information available about the impact of available treatment sequences on clinical outcomes and costs.

Methods: A budget impact model was developed to represent the current treatment pathways for relapsed low-grade or follicular B-cell non-Hodgkin lymphoma. First-line treatment regimens were selected based on the Category 1 recommendations from the National Comprehensive Cancer Network guidelines for follicular lymphoma. Those included were the most commonly used: BR, RCHOP, and RCVP. For purposes of this model, the second-line regimens included Y90 ibritumomab (Y90), rituximab monotherapy with 4 weekly doses, and idelalisib. Treatment outcomes were based on the published literature that summarized the overall response rate, median duration of response (DOR), and toxicity. Total costs per treatment regimen were based on medical care, drugs administered, and associated adverse effects. The 2015 Medicare fee schedule rates were used to estimate the medical care costs. Total DOR and costs were determined for each treatment regimen.

Results: The treatment sequence, BR followed by Y90 at the time of relapse, had the longest predicted DOR (7.2 years). The associated treatment sequence costs were $157,712 for BR followed by Y90, compared with $224,628 and $145,047 for BR followed by idelalisib and BR followed by rituximab monotherapy, respectively, with a predicted DOR of 6.7 years. The predicted DOR for treatment sequences starting with RCVP and RCHOP and followed by Y90 was about 3 years less than BR followed by Y90 at a cost increase of $11,102 and $37,866, respectively. The use of idelalisib after RCVP or RCHOP had a predicted DOR of 3.6 years, respectively, with associated costs that were greater than BR followed by Y90.

Conclusions: The use of Y90 ibritumomab after BR demonstrated the optimal patient outcomes at one of the lowest cost profiles.

Backround: Mastocytosis is a rare disease with a variable clinical course. Pure red cell aplasia has not been frequently reported in association with aggressive systemic mastocytosis. CD30 is often expressed on mast cells in systemic mastocytosis. The CD30-directed antibody-drug conjugate brentuximab vedotin has shown in vitro activity against CD30-positive neoplastic mast cells. We describe a case of systemic mastocytosis with the development of severe hemolytic anemia, pure red cell aplasia, and evidence of weak bone marrow CD30 positivity. Brentuximab vedotin was administered with some evidence of clinical benefit.

Patient: A 64-year-old man presented with fatigue and a 20 pound weight loss. He was found to have transfusion-dependent anemia, a low reticulocyte count, and splenomegaly. Evaluation for gastrointestinal blood loss was unremarkable. Bone marrow biopsy showed mast cell aggregates, spindle-shaped mast cells, CD-2 and CD25-positivity, increased myelopoiesis and dysmegakaryopoiesis, red cell aplasia, eosinophilia, and increased reticulin fibrosis. The D816V c-kit mutation was present, and serum tryptase level was elevated at 114 ng/mL. He later developed Coombs-positive hemolysis and direct hyperbilirubinemia, concerning for mast cell liver infiltration.

Intervention: The patient received high-dose steroids and red blood cell transfusions. Interferon alpha and cladribine were contraindicated, given severe liver dysfunction. He was started on hydroxyurea. He received a dose of brentuximab vedotin 1.8 mg/kg IV. Thirteen days after starting steroids and 10 days after brentuximab, haptoglobin became detectable, and total bilirubin decreased to 18.4 mg/dL from 40.5 mg/dL. Red blood cell transfusion requirements decreased. He then developed severe neutropenia, pneumonia with sepsis, and respiratory failure, requiring intubation, maximal pressor support, and broad-spectrum antibiotics. Despite these measures, he expired.

Discussion/Conclusion: This is a rare case of pure red cellaplasia in the setting of aggressive systemic mastocytosis. Therapeutic options were limited in the setting of liver dysfunction, and therapy with brentuximab vedotin resulted in initial clinical benefit. Brentuximab vedotin is being investigated in patients with advanced systemic mastocytosis or mast cell leukemia.

Backround: Mastocytosis is a rare disease with a variable clinical course. Pure red cell aplasia has not been frequently reported in association with aggressive systemic mastocytosis. CD30 is often expressed on mast cells in systemic mastocytosis. The CD30-directed antibody-drug conjugate brentuximab vedotin has shown in vitro activity against CD30-positive neoplastic mast cells. We describe a case of systemic mastocytosis with the development of severe hemolytic anemia, pure red cell aplasia, and evidence of weak bone marrow CD30 positivity. Brentuximab vedotin was administered with some evidence of clinical benefit.

Patient: A 64-year-old man presented with fatigue and a 20 pound weight loss. He was found to have transfusion-dependent anemia, a low reticulocyte count, and splenomegaly. Evaluation for gastrointestinal blood loss was unremarkable. Bone marrow biopsy showed mast cell aggregates, spindle-shaped mast cells, CD-2 and CD25-positivity, increased myelopoiesis and dysmegakaryopoiesis, red cell aplasia, eosinophilia, and increased reticulin fibrosis. The D816V c-kit mutation was present, and serum tryptase level was elevated at 114 ng/mL. He later developed Coombs-positive hemolysis and direct hyperbilirubinemia, concerning for mast cell liver infiltration.

Intervention: The patient received high-dose steroids and red blood cell transfusions. Interferon alpha and cladribine were contraindicated, given severe liver dysfunction. He was started on hydroxyurea. He received a dose of brentuximab vedotin 1.8 mg/kg IV. Thirteen days after starting steroids and 10 days after brentuximab, haptoglobin became detectable, and total bilirubin decreased to 18.4 mg/dL from 40.5 mg/dL. Red blood cell transfusion requirements decreased. He then developed severe neutropenia, pneumonia with sepsis, and respiratory failure, requiring intubation, maximal pressor support, and broad-spectrum antibiotics. Despite these measures, he expired.

Discussion/Conclusion: This is a rare case of pure red cellaplasia in the setting of aggressive systemic mastocytosis. Therapeutic options were limited in the setting of liver dysfunction, and therapy with brentuximab vedotin resulted in initial clinical benefit. Brentuximab vedotin is being investigated in patients with advanced systemic mastocytosis or mast cell leukemia.

Backround: Mastocytosis is a rare disease with a variable clinical course. Pure red cell aplasia has not been frequently reported in association with aggressive systemic mastocytosis. CD30 is often expressed on mast cells in systemic mastocytosis. The CD30-directed antibody-drug conjugate brentuximab vedotin has shown in vitro activity against CD30-positive neoplastic mast cells. We describe a case of systemic mastocytosis with the development of severe hemolytic anemia, pure red cell aplasia, and evidence of weak bone marrow CD30 positivity. Brentuximab vedotin was administered with some evidence of clinical benefit.

Patient: A 64-year-old man presented with fatigue and a 20 pound weight loss. He was found to have transfusion-dependent anemia, a low reticulocyte count, and splenomegaly. Evaluation for gastrointestinal blood loss was unremarkable. Bone marrow biopsy showed mast cell aggregates, spindle-shaped mast cells, CD-2 and CD25-positivity, increased myelopoiesis and dysmegakaryopoiesis, red cell aplasia, eosinophilia, and increased reticulin fibrosis. The D816V c-kit mutation was present, and serum tryptase level was elevated at 114 ng/mL. He later developed Coombs-positive hemolysis and direct hyperbilirubinemia, concerning for mast cell liver infiltration.

Intervention: The patient received high-dose steroids and red blood cell transfusions. Interferon alpha and cladribine were contraindicated, given severe liver dysfunction. He was started on hydroxyurea. He received a dose of brentuximab vedotin 1.8 mg/kg IV. Thirteen days after starting steroids and 10 days after brentuximab, haptoglobin became detectable, and total bilirubin decreased to 18.4 mg/dL from 40.5 mg/dL. Red blood cell transfusion requirements decreased. He then developed severe neutropenia, pneumonia with sepsis, and respiratory failure, requiring intubation, maximal pressor support, and broad-spectrum antibiotics. Despite these measures, he expired.

Discussion/Conclusion: This is a rare case of pure red cellaplasia in the setting of aggressive systemic mastocytosis. Therapeutic options were limited in the setting of liver dysfunction, and therapy with brentuximab vedotin resulted in initial clinical benefit. Brentuximab vedotin is being investigated in patients with advanced systemic mastocytosis or mast cell leukemia.

CHICAGO – Statin use is associated with a decrease in the prevalence of skeletal-related events in patients with stage I multiple myeloma, findings from a review of 120 cases suggest.

The prevalence of skeletal-related events (SREs) was 35% in 54 patients who had been treated with statins, compared with 56% in 66 statin-naive patients. The difference between the groups remained significant after adjusting for bisphosphonate use, calcium and vitamin D supplementation, history of osteoporosis, and second malignancy, Dr. Fernando Vargas of the University of Miami reported in a poster at the American Society of Hematology Meeting on Hematologic Malignancies.

Study subjects were adults with multiple myeloma seen at a single institution between 2007 and 2012. Those with statin use prior to the development of SREs – defined as pathologic fractures, necessity of orthopedic intervention, radiation therapy, or spinal cord compression – were included. Patients with smoldering myeloma or monoclonal gammopathy of indeterminate significance were excluded.

The statin-experienced and statin-naive groups were similar with respect to smoking history, alcohol abuse, documented diagnosis of osteoporosis, coexistent malignancy, hypothyroidsim, Paget’s disease of the bone, and use of bisphosphonates or calcium plus vitamin D supplementation. The groups also were similar with respect to distribution of cancer staging, and after correcting for International Surgical Staging stage, the SRE risk reduction associated with statin use was significant only in patients with stage I multiple myeloma. One patient with stage I disease (10%) in the statin-experienced group developed SREs, compared with 6 (62%) in the statin-naive group, Dr. Vargas said.

SREs are associated with significant morbidity and mortality in patients with multiple myeloma, and the results of studies assessing the effect of statins on overall disease response in myeloma patients undergoing chemotherapy have been conflicting, he explained, noting that “this analysis was proposed on the grounds that statins inhibit the mevalonic acid pathway upstream from the site of action of bisphosphonates (i.e. famesyl diphosphate synthase blockade), an integral part of multiple myeloma supportive treatment.”

He and his colleagues hypothesized that, given their site of action, statins would have bone protective effects.

Indeed, statins were found to be associated with a decrease in the prevalence of SREs in patients with stage I multiple myeloma, he said.

“Given the well documented safety profile of statins and their ease of use, we suggest that a prospective study be done to further assess their role in the prevention of SREs in multiple myeloma patients,” he concluded, noting that the findings in ISS stage I patients also suggest a possible role for statin use in patients with high-risk smoldering myeloma.

Dr. Vargas reported having no disclosures.

sworcester@frontlinemedcom.com

CHICAGO – Statin use is associated with a decrease in the prevalence of skeletal-related events in patients with stage I multiple myeloma, findings from a review of 120 cases suggest.

The prevalence of skeletal-related events (SREs) was 35% in 54 patients who had been treated with statins, compared with 56% in 66 statin-naive patients. The difference between the groups remained significant after adjusting for bisphosphonate use, calcium and vitamin D supplementation, history of osteoporosis, and second malignancy, Dr. Fernando Vargas of the University of Miami reported in a poster at the American Society of Hematology Meeting on Hematologic Malignancies.

Study subjects were adults with multiple myeloma seen at a single institution between 2007 and 2012. Those with statin use prior to the development of SREs – defined as pathologic fractures, necessity of orthopedic intervention, radiation therapy, or spinal cord compression – were included. Patients with smoldering myeloma or monoclonal gammopathy of indeterminate significance were excluded.

The statin-experienced and statin-naive groups were similar with respect to smoking history, alcohol abuse, documented diagnosis of osteoporosis, coexistent malignancy, hypothyroidsim, Paget’s disease of the bone, and use of bisphosphonates or calcium plus vitamin D supplementation. The groups also were similar with respect to distribution of cancer staging, and after correcting for International Surgical Staging stage, the SRE risk reduction associated with statin use was significant only in patients with stage I multiple myeloma. One patient with stage I disease (10%) in the statin-experienced group developed SREs, compared with 6 (62%) in the statin-naive group, Dr. Vargas said.

SREs are associated with significant morbidity and mortality in patients with multiple myeloma, and the results of studies assessing the effect of statins on overall disease response in myeloma patients undergoing chemotherapy have been conflicting, he explained, noting that “this analysis was proposed on the grounds that statins inhibit the mevalonic acid pathway upstream from the site of action of bisphosphonates (i.e. famesyl diphosphate synthase blockade), an integral part of multiple myeloma supportive treatment.”

He and his colleagues hypothesized that, given their site of action, statins would have bone protective effects.

Indeed, statins were found to be associated with a decrease in the prevalence of SREs in patients with stage I multiple myeloma, he said.

“Given the well documented safety profile of statins and their ease of use, we suggest that a prospective study be done to further assess their role in the prevention of SREs in multiple myeloma patients,” he concluded, noting that the findings in ISS stage I patients also suggest a possible role for statin use in patients with high-risk smoldering myeloma.

Dr. Vargas reported having no disclosures.

sworcester@frontlinemedcom.com

CHICAGO – Statin use is associated with a decrease in the prevalence of skeletal-related events in patients with stage I multiple myeloma, findings from a review of 120 cases suggest.

The prevalence of skeletal-related events (SREs) was 35% in 54 patients who had been treated with statins, compared with 56% in 66 statin-naive patients. The difference between the groups remained significant after adjusting for bisphosphonate use, calcium and vitamin D supplementation, history of osteoporosis, and second malignancy, Dr. Fernando Vargas of the University of Miami reported in a poster at the American Society of Hematology Meeting on Hematologic Malignancies.

Study subjects were adults with multiple myeloma seen at a single institution between 2007 and 2012. Those with statin use prior to the development of SREs – defined as pathologic fractures, necessity of orthopedic intervention, radiation therapy, or spinal cord compression – were included. Patients with smoldering myeloma or monoclonal gammopathy of indeterminate significance were excluded.

The statin-experienced and statin-naive groups were similar with respect to smoking history, alcohol abuse, documented diagnosis of osteoporosis, coexistent malignancy, hypothyroidsim, Paget’s disease of the bone, and use of bisphosphonates or calcium plus vitamin D supplementation. The groups also were similar with respect to distribution of cancer staging, and after correcting for International Surgical Staging stage, the SRE risk reduction associated with statin use was significant only in patients with stage I multiple myeloma. One patient with stage I disease (10%) in the statin-experienced group developed SREs, compared with 6 (62%) in the statin-naive group, Dr. Vargas said.

SREs are associated with significant morbidity and mortality in patients with multiple myeloma, and the results of studies assessing the effect of statins on overall disease response in myeloma patients undergoing chemotherapy have been conflicting, he explained, noting that “this analysis was proposed on the grounds that statins inhibit the mevalonic acid pathway upstream from the site of action of bisphosphonates (i.e. famesyl diphosphate synthase blockade), an integral part of multiple myeloma supportive treatment.”

He and his colleagues hypothesized that, given their site of action, statins would have bone protective effects.

Indeed, statins were found to be associated with a decrease in the prevalence of SREs in patients with stage I multiple myeloma, he said.

“Given the well documented safety profile of statins and their ease of use, we suggest that a prospective study be done to further assess their role in the prevention of SREs in multiple myeloma patients,” he concluded, noting that the findings in ISS stage I patients also suggest a possible role for statin use in patients with high-risk smoldering myeloma.

Dr. Vargas reported having no disclosures.

sworcester@frontlinemedcom.com

Purpose: Review the clinical presentation and pathophysiology of hypercalcemia in diffuse large B-cell lymphoma.

Methods: Case study of a 59-year-old man with untreateddiffuse large B-cell lymphoma (DLBCL) who presented with anorexia, constipation, and weight loss in the setting of a fungating breast mass.

Results: The patient presented to an outside hospital 1.5 years prior for a left breast mass biopsy that was diagnosed as triple-hit DLBCL. However, he deferred treatment at that time. He subsequently experienced anorexia, nausea, vomiting, consti-pation, and a 20-pound unintentional weight loss. At hospital presentation, a physical examination revealed enlargement of the left breast mass measuring 8 x 14 cm with central ulceration and malodorous purulent discharge and a proximal 7 x 4 cm firm axillary mass. The patient reported no abdominal pain or fever. He was found to have acute kidney injury (creatinine 2.9 mg/dL); hyperuricemia (13.3 mg/dL); and corrected hypercalcemia (17.4 mg/dL). Empiric treatment for tumor lysis syndrome with rasburicase, allopurinol, and aggressive IV hydration was initiated. Further evaluation revealed normal lactate dehydrogenase and K+.The patient was found to have an elevated vitamin D 1,25-OH (244 pg/mL) but low parathyroid hormone (PTH) (6.4 pg/mL); normal PTH-rP; mild hyperphosphatemia (5.5 mg/dL); and negative serum protein electrophoresis/urine protein electrophoresis. Clinical presentation was suggestive of vitamin D-mediated hypercalcemia. The patient received pamidronate and aggressive IV hydration. Electrolyte abnormalities and renal function gradually improved. Computed tomography and positron emission tomography imaging revealed masses localized to the left chest wall with ipsilateral axillary lymphadenopathy. Surgical biopsy of the mass reconfirmed DLBCL ultimately staged as IIE. Following stabilization, the patient declined more aggressive chemotherapy and received R-CHOP with a complete response.

Conclusions: The patient presented with vitamin D-mediated hypercalcemia in the setting of aggressive triple-hit DLBCL. Vitamin D-mediated hypercalcemia occurs in < 1% of lymphomas, and < 0.5% of breast tumors are primary lymphomas. This case study presents a rare constellation of findings at diagnosis. The patient responded to IV hydration and pamidronate and subsequently received R-CHOP for stage IIE DLBCL. While hypercalcemia in the setting of malignancy is associated with poor prognosis, treatment of hypercalcemia does not improve survival.

Purpose: Review the clinical presentation and pathophysiology of hypercalcemia in diffuse large B-cell lymphoma.

Methods: Case study of a 59-year-old man with untreateddiffuse large B-cell lymphoma (DLBCL) who presented with anorexia, constipation, and weight loss in the setting of a fungating breast mass.

Results: The patient presented to an outside hospital 1.5 years prior for a left breast mass biopsy that was diagnosed as triple-hit DLBCL. However, he deferred treatment at that time. He subsequently experienced anorexia, nausea, vomiting, consti-pation, and a 20-pound unintentional weight loss. At hospital presentation, a physical examination revealed enlargement of the left breast mass measuring 8 x 14 cm with central ulceration and malodorous purulent discharge and a proximal 7 x 4 cm firm axillary mass. The patient reported no abdominal pain or fever. He was found to have acute kidney injury (creatinine 2.9 mg/dL); hyperuricemia (13.3 mg/dL); and corrected hypercalcemia (17.4 mg/dL). Empiric treatment for tumor lysis syndrome with rasburicase, allopurinol, and aggressive IV hydration was initiated. Further evaluation revealed normal lactate dehydrogenase and K+.The patient was found to have an elevated vitamin D 1,25-OH (244 pg/mL) but low parathyroid hormone (PTH) (6.4 pg/mL); normal PTH-rP; mild hyperphosphatemia (5.5 mg/dL); and negative serum protein electrophoresis/urine protein electrophoresis. Clinical presentation was suggestive of vitamin D-mediated hypercalcemia. The patient received pamidronate and aggressive IV hydration. Electrolyte abnormalities and renal function gradually improved. Computed tomography and positron emission tomography imaging revealed masses localized to the left chest wall with ipsilateral axillary lymphadenopathy. Surgical biopsy of the mass reconfirmed DLBCL ultimately staged as IIE. Following stabilization, the patient declined more aggressive chemotherapy and received R-CHOP with a complete response.

Conclusions: The patient presented with vitamin D-mediated hypercalcemia in the setting of aggressive triple-hit DLBCL. Vitamin D-mediated hypercalcemia occurs in < 1% of lymphomas, and < 0.5% of breast tumors are primary lymphomas. This case study presents a rare constellation of findings at diagnosis. The patient responded to IV hydration and pamidronate and subsequently received R-CHOP for stage IIE DLBCL. While hypercalcemia in the setting of malignancy is associated with poor prognosis, treatment of hypercalcemia does not improve survival.

Purpose: Review the clinical presentation and pathophysiology of hypercalcemia in diffuse large B-cell lymphoma.

Methods: Case study of a 59-year-old man with untreateddiffuse large B-cell lymphoma (DLBCL) who presented with anorexia, constipation, and weight loss in the setting of a fungating breast mass.

Results: The patient presented to an outside hospital 1.5 years prior for a left breast mass biopsy that was diagnosed as triple-hit DLBCL. However, he deferred treatment at that time. He subsequently experienced anorexia, nausea, vomiting, consti-pation, and a 20-pound unintentional weight loss. At hospital presentation, a physical examination revealed enlargement of the left breast mass measuring 8 x 14 cm with central ulceration and malodorous purulent discharge and a proximal 7 x 4 cm firm axillary mass. The patient reported no abdominal pain or fever. He was found to have acute kidney injury (creatinine 2.9 mg/dL); hyperuricemia (13.3 mg/dL); and corrected hypercalcemia (17.4 mg/dL). Empiric treatment for tumor lysis syndrome with rasburicase, allopurinol, and aggressive IV hydration was initiated. Further evaluation revealed normal lactate dehydrogenase and K+.The patient was found to have an elevated vitamin D 1,25-OH (244 pg/mL) but low parathyroid hormone (PTH) (6.4 pg/mL); normal PTH-rP; mild hyperphosphatemia (5.5 mg/dL); and negative serum protein electrophoresis/urine protein electrophoresis. Clinical presentation was suggestive of vitamin D-mediated hypercalcemia. The patient received pamidronate and aggressive IV hydration. Electrolyte abnormalities and renal function gradually improved. Computed tomography and positron emission tomography imaging revealed masses localized to the left chest wall with ipsilateral axillary lymphadenopathy. Surgical biopsy of the mass reconfirmed DLBCL ultimately staged as IIE. Following stabilization, the patient declined more aggressive chemotherapy and received R-CHOP with a complete response.

Conclusions: The patient presented with vitamin D-mediated hypercalcemia in the setting of aggressive triple-hit DLBCL. Vitamin D-mediated hypercalcemia occurs in < 1% of lymphomas, and < 0.5% of breast tumors are primary lymphomas. This case study presents a rare constellation of findings at diagnosis. The patient responded to IV hydration and pamidronate and subsequently received R-CHOP for stage IIE DLBCL. While hypercalcemia in the setting of malignancy is associated with poor prognosis, treatment of hypercalcemia does not improve survival.