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.

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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.

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.

A 6-year-old girl had a group A beta-haemolytic streptococcal (GABS) throat infection and Henoch-Schonlein purpura (HSP). The clinical course was complicated by nephrotic syndrome due to crescentic glomerulonephritis, transient neurological symptoms due to focal ischaemia of the brain, and congestive cardiac failure due to myocarditis. The clinical presentation highlights the diversity of systemic involvement in HSP, the transient nature of apparently serious central nervous system involvement, and a possible role of GABS in its aetiology.

One of the principal targets of HIV infection is the human peripheral blood CD4+ T cell, resulting in progressive CD4+ lymphocyte loss. Hypothesized mechanisms for this loss include apoptosis, cytolytic reactions, V-beta gene deletion of the T-cell receptor (TCR) by superantigens, CD4+ lymphocyte syncytium formation, and autoimmune reactions. In adults with HIV infection, the critical decline in CD4+ lymphocyte number that heralds the onset of AIDS-defining conditions is well characterized, whereas in infants and children the critical level of CD4+ cells predisposing to the development of AIDS-defining conditions or mortality is not fully characterized, due to an incomplete knowledge of CD4+ lymphocyte number and changes with age in normal and HIV-infected children. In a prospective study of 317 infants born to HIV-infected women, early results show that the monthly change in absolute CD4+ lymphocyte number over a 3- to 9-month period in HIV-infected infants was -109 cells/mm3 per month, at least double the rate of decline measured in HIV-noninfected infants in the study or that calculated from normal infants' values reported in the literature. In other clinical studies in HIV-infected infants and children, it was possible to study the effect of low CD4+ cell counts on clinical status and mortality. In HIV-infected pediatric patients younger than 1 year, it was possible to correlate low CD4+ cell number with advanced disease status (CDC pediatric class P-2). It was also possible to correlate extremely low CD4+ cell counts (< 200 cells/mm3) in HIV-infected children with a significant risk of mortality within the next 3 months of life. Sequential CD4+ cell analysis of HIV-high-risk infants will delineate the rate of HIV-related decline in CD4+ cells, thus facilitating the diagnosis of HIV infection and aiding in identification of HIV-infected children at high risk of disease progression or death.

Anemia is a common complication in end-stage renal disease (ESRD) patients. On the other hand, idiopathic erythrocytosis is extremely rare, with only a few cases reported in the literature. We present a case of erythrocytosis that developed after initiating hemodialysis. A 68-year-old male with a history of ESRD secondary to diabetes presented with erythrocytosis that started a few months after initiating dialysis in the absence of having received erythropoietin-stimulating agents or iron supplements. His erythropoietin level was elevated, with a negative JAK2 mutation. Blood gases showed normal oxygen and CO₂, with slightly elevated carboxyhemoglobin. Tiny foci in both kidneys were noted, representing vascular calcifications or renolithiasis. There was no radiological evidence of neoplasms or cysts. After excluding secondary causes, a diagnosis of idiopathic erythrocytosis was made. The patient underwent intermittent phlebotomies during dialysis, and his hemoglobin went from 18.5 to 14 mg/dl. Erythrocytosis in ESRD patients is very rare. So far, there is no complete understanding of the underlying pathophysiology; however, there seem to be multiple possible reasons for an increased erythropoietin level. Phlebotomy is a successful and easy way to control erythrocytosis in such patients. Angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, currently being used in posttransplant erythrocytosis, might also be considered.

e19234 Background: More sophisticated treatment techniques in cancer management have led to increased complexity in patient care. The perception of cancer outcomes is different between physicians and may result in premature hospice referral in hospitalized patients with or without cancer-related acute illness. We created a clinical scenario-based survey to assess perceptions of goals of care choices of inpatient primary providers while caring for patients with malignancies. Methods: A survey was developed and sent to all internal medicine and primary care residency programs nationwide via “Survey Monkey” online platform. Cases were adapted from real patient encounters. A total of 8 cases were presented and physicians were asked to determine level of care. Two cases represented potentially curable disease, three described patients with treatable but incurable malignancies, and the remainder described patients with advanced malignancies appropriate for hospice care. Physicians were also questioned about patterns of consulting hematology/oncology (HO) and their opinions regarding barriers in providing care to patients with malignancies. Results: Of 269 physicians who took the survey, 184 physicians (68%) fully completed it. 37% reported consulting HO for every patient with underlying malignancy, while 2% of the providers do not have access to HO consultants. In curable cancers, 65% of physicians would request inpatient oncology consultation while 11% would initiate hospice referral. There was a significant variation in choices for care in treatable cancer cases. In case 1, 13% of surveyed physicians thought that patients were hospice appropriate, while a higher percentage felt the same for the 2nd and 3rd cases with 18% and 52%, respectively. For hospice-appropriate patients, 25% of providers felt that further cancer treatment should be performed in younger patients, while the majority agreed on hospice referral (91%) for older patients. The most reported barrier to providing care to hospitalized patients with malignancies was a disconnect between the perception of goals of care of the primary treating oncologist/hematologist and patient’s actual condition. Conclusions: Perceptions about goals of care in hospitalized patients with malignancies varies and is affected by patient age and perceived severity of disease, which doesn’t necessarily translate into outcome statistics reported in literature. A multidisciplinary approach and treating oncologist involvement might be warranted when patient goals of care change is planned in hospital setting.