Myonecrosis in Sickle Cell Anemia—Overlooked and Underdiagnosed

Medical literature detailing muscular complications of sickle cell anemia is sparse and limited to a few case-reports. Features consistent with myositis and myonecrosis are often overlooked and patients are inadequately treated, leading to unforeseen complications. We report an interesting case of sickle cell myonecrosis and review the existing literature on this subject

Adult T-Cell Leukemia/Lymphoma with CLL-Like Morphology—A Case Report

Adult T-cell Leukemia/Lymphoma (ATL) is rarely seen in the U.S. and Europe, usually limited to African Americans from the southeastern U.S. and immigrants from HTLV-1 endemic areas. Reaching an accurate and timely diagnosis of ATL in such nonendemic areas can be challenging, owing to limited exposure, diverse manifestations, and varying cell morphology. We present a case of chronic adult T-cell leukemia (ATL) with Chronic Lymphocytic Leukemia- (CLL-) like morphology that remained untreated for ten years and then developed treatment refractory acute ATL crisis

New targets for the treatment of follicular lymphoma

The last two decades have witnessed striking advances in our understanding of the biological factors underlying the development of Follicular lymphoma (FL). Development of newer treatment approaches have improved the outlook for many individuals with these disorders; however, with these advances come new questions. Given the long-term survival of patients with FL, drugs with favourable side-effect profile and minimal long-term risks are desired. FL is incurable with current treatment modalities. It often runs an indolent course with multiple relapses and progressively shorter intervals of remission. The identification of new targets and development of novel targeted therapies is imperative to exploit the biology of FL while inherently preventing relapse and prolonging survival. This review summarizes the growing body of knowledge regarding novel therapeutic targets, enabling the concept of individualized targeted therapy for the treatment of FL

Brain metastasis in patients with adrenocortical carcinoma: a clinical series.

INTRODUCTION: Adrenocortical carcinoma (ACC) is a heterogeneous and rare disease. At presentation or at the time of a recurrence, the disease commonly spreads to the liver, lungs, lymph nodes, and bones. The brain has only rarely been reported as a site of metastases. OBJECTIVE: The aims of this report were to describe the clinical characteristics of patients with ACC who developed brain metastasis and were evaluated at the National Cancer Institute. METHODS: We describe the history and clinical presentation of six patients with ACC and metastatic disease in the brain. Images of the six patients and pathology slides were reviewed when available. RESULTS: The median age at the time of the diagnosis of ACC was 42 years. The median time from the initial diagnosis until the presentation of brain metastasis was 43 months. As a group the patients had previously received multiples lines of chemotherapy (median of three), and they presented with one to three metastatic brain lesions. Four patients underwent metastasectomy, one had radiosurgery, and one had both modalities. Two patients are still alive, three died, between 2 and 14 months after the diagnosis of brain metastases, and one was lost to follow-up. CONCLUSION: Patients with advanced ACC can rarely present with metastasis to the brain, most often long after the initial diagnosis. Timely diagnosis of brain metastasis with appropriate intervention after discussion in a multidisciplinary meeting can improve the prognosis in this particular scenario

Utility Of An Algorithm For Use Of Plerixafor In Filgrastim-based Hematopoietic Progenitor Cell Mobilization In Patients With Plasma Cell Myeloma Treated With Carfilzomib-Lenalidomide-Dexamethasone

Abstract Mobilization of hematopoietic progenitor cells (HPC) for subsequent autologous transplantation is difficult in patients with plasma cell myeloma (PCM) due to poor marrow reserve. Targeted HPC yields are generally not achieved in a single apheresis procedure without use of plerixafor as a supplement to standard filgrastim. Strategies to limit use of plerixafor, due to its expense, to cases of poor CD34 mobilization have been developed, but their applicability in patients receiving the novel induction regimen carfilzomib-lenalidomide-dexamethasone (CRD) (Blood 2012;120:1801) has not been described. We prospectively studied the CD34 cell mobilization responses of PCM patients following CRD induction, using a CD34 cell predictive algorithm to determine when plerixafor should be added to the mobilization regimen. Thirty patients, including 23 with PCM and 7 with smoldering PCM, mean age 55 (range 40-72), 47% male, received 4 to 7 cycles of CRD (median, 5 cycles), with the last dose of lenalidomide given at least one week prior to mobilization. Filgrastim 10-16 mcg/kg/day was given as a single evening dose for 5 days, with circulating CD34 count assessed 12 hours after the 4th dose. The pre-apheresis CD34 count after the 5th dose of filgrastim was predicted to be 10% greater than that after the 4th dose; this prediction was validated with an actual pre-apheresis CD34 count obtained the following day. Prior mobilization data derived from healthy HPC apheresis donors was used to generate a regression formula, y=0.45x+0.86, where x=the pre-apheresis circulating CD34 count after the 5th dose of filgrastim, and y=the expected yield of the apheresis procedure, expressed as millions of CD34 cells harvested per liter processed. Targeted yield was ≥ 4 x 106 CD34 cells/kg, with minimum acceptable yield ≥ 2 x 106 CD34 cells/kg. Plerixafor 240 mcg/kg was given with the 5th dose of filgrastim, 8-10 hours prior to apheresis, if the regression equation predicted a CD34 cell yield of < 4 x 106 CD34 cells/kg in a single procedure with a maximum of 30 liters processed. The actual volume processed was based on the stat blood CD34 count drawn immediately prior to apheresis. Procedures were performed on the Cobe Spectra device; continuous intravenous calcium was used to mitigate citrate toxicity. Central lines were required in 67% of subjects. Mean CD34 cell count in the entire group after the 4th dose of filgrastim was 29/uL (range 2-88/uL). Using the regression formula as a guide, 17/30 (57%) of patients received plerixafor. CD34 counts increased 4.2-fold in patients receiving plerixafor, from 15 ± 9/uL (m ± SD) on the day prior to apheresis to 53 ± 30/uL immediately pre-apheresis; CD34 counts did not change in patients who received filgrastim alone (from 48 ± 17/uL to 45 ± 19/uL). Guided by the stat pre-apheresis CD34 count, the volume processed in the first apheresis procedure was the same, 23 ± 7 (range 12-30) liters, with or without plerixafor. CD34 cells were collected with 72 ± 14% efficiency. First-procedure CD34 cell yields were 6.4 ± 2.5 x 106/kg (range 2.5-10.1) with supplemental plerixafor vs 5.8 ± 2.5 x 106/kg (range 1.1-9.3) with filgrastim alone. Only 2/30 patients underwent a second procedure; neither received plerixafor prior to the first procedure, and both received it prior to the second. In one patient, criteria for plerixafor administration were met, but the drug was inadvertently not given prior to the first procedure; in the second patient, an unexpectedly low pre-apheresis CD34 count was traced to inadequate self-administration of the 5th dose of filgrastim. All 30 patients achieved the minimum CD34 collection goal of ≥ 2 x 106 cells/kg and 29/30 did this in one procedure. The higher targeted collection goal of ≥ 4 x 106 CD34 cells/kg was achieved in a single procedure by 76% of patients in both the plerixafor group and the filgrastim-alone group. There was a trend for higher cumulative lenalidomide and carfilzomib doses to be associated with lower CD34 mobilization responses to filgrastim. Induction treatment with CRD does not appear to impair HPC mobilization response to filgrastim in patients with PCM, compared to older regimens. An algorithm that uses the CD34 cell count after 4 doses of filgrastim to predict the following day’s pre-apheresis CD34 count and thus determine whether plerixafor supplementation is needed, was useful in identifying the 40% of CRD-treated myeloma patients who do not need plerixafor. Disclosures: No relevant conflicts of interest to declare