Blast phase in chronic myelogenous leukemia (CML) has rarely been reported to involve extramedullary sites like skin, lymph nodes, and central nervous system. Clinical history, characteristic hematologic findings (elevated leukocyte counts, myelocytic predominance, and basophilia), and Philadelphia chromosome are of high diagnostic significance especially in isolated extramedullary presentations. We describe a unique case of CML relapse with blast phase involving the eye. A 66-year-old man with a known diagnosis of CML on imatinib and in molecular remission for 3 years presented with a painful blind eye. Histologic examination revealed diffuse involvement of choroid, iris, vitreous humor, and the optic nerve by blast cells. The blasts expressed CD34, aberrant TdT, and a myeloid phenotype (CD13, CD33, and CD117). Fluorescence in situ hybridization (FISH) of vitreous fluid detected BCR-ABL1 gene rearrangement. Additionally, trisomy 8 and gains of 9 and 22 were seen which were not present in the initial diagnostic marrow study 3 years ago. At relapse, the bone marrow, peripheral blood, and the cerebrospinal fluid were not involved by CML. Patient received induction chemotherapy and single dose prophylactic intrathecal methotrexate and was maintained on antityrosine kinase therapy and eventually underwent allogenic stem cell transplantation.
Sideroblastic anemia secondary to zinc toxicity A 52-year-old African-American woman with a history of allergic rhinitis and alcohol abuse presented with a presyncope. The following levels were found: hemoglobin, 3.7 g/dL; mean cell volume, 82 fL; white blood cell count, 9.2 3 10 3 /mL; and platelets, 168 3 10 3 /mL. The workup was negative for hemolysis or bleeding. Her blood alcohol level was normal. B 12 and folate levels were normal. A peripheral smear showed dimorphic red blood cells with Pappenheimer bodies (panel A). A bone marrow biopsy showed slightly hypercellular marrow with intact trilineage hematopoiesis and mild erythroid dyspoiesis. An iron stain showed adequate iron content and occasional ring sideroblasts (panel B). Marrow morphology and cytogenetic analysis did not support a myelodysplastic syndrome. Copper and ceruloplasmin levels were low at 515 mg/L and 16 mg/ dL (normal ranges, 810-1990 mg/L and 20-60 mg/dL), respectively. The zinc level was elevated at 186 mg/dL (normal range, 60-130 mg/dL). The patient admitted to self-treating her chronic cough with zinc lozenges for the last 4 months.Our impression was that sideroblastic anemia caused by copper deficiency induced by zinc lozenge use was a significant component of the severe anemia. Zinc lozenges were discontinued, and copper supplements were initiated, with complete hematologic recovery in 4 weeks. For additional images, visit the ASH IMAGE BANK, a reference and teaching tool that is continually updated with new atlas and case study images. For more information visit http://imagebank.hematology.org.
Molecular studies have shown metformin to have a promising effect in lymphoma; however, there is lack of studies translating this effect into clinical settings. This was a case-control study to assess the clinical effect of metformin in diabetic diffuse large B-cell lymphoma (DLBCL) patients. Case subjects were diabetic on metformin with a new diagnosis of DLBCL. A total of 24 case subjects were identified, and for each case a control was matched. Outcomes of this study were to assess overall response rate, complete remission rate, progression free survival, and overall survival between the two groups. There was a significant increase in overall response rate, complete remission rate, and improved progression free survival in the metformin group compared to the control group, however, no significant overall survival difference was observed. Metformin use might be associated with an improved response rates and progression-free survival in diabetic DLBCL patients.
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