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Pulmonary hypertension in sickle cell disease (SCD) is a complex phenomenon resulting from multiple overlapping etiologies, including pulmonary vasoconstriction in the setting of chronic hemolytic anemia, diastolic dysfunction, and chronic thromboembolic disease. The presence of pulmonary hypertension of any cause in SCD confers a significant increase in mortality risk. Evidence to guide the management of patients with sickle cell disease and chronic thromboembolic pulmonary hypertension (CTEPH) is scant and largely the realm of case reports and small case series. Centered on a discussion of a complex young patient with hemoglobin hemoglobin SC who ultimately underwent treatment with pulmonary thromboendarterectomy, we review the available literature to guide management and discuss and overview of treatment of CTEPH in SCD, considering the unique considerations and challenges facing patients suffering from this multisystem disease. [ABSTRACT FROM AUTHOR]

This article discusses a case study of a 51-year-old woman with systemic lupus erythematosus (SLE) who developed macrophage activation syndrome (MAS) after receiving granulocyte colony-stimulating factor (G-CSF) treatment for neutropenia. MAS is a potentially life-threatening complication characterized by excessive immune system activation. The patient's clinical course strongly suggests that G-CSF administration was the trigger for MAS. The article highlights the need for clinicians to be aware of the potential complications associated with G-CSF administration in SLE patients. [Extracted from the article]

Summary: In the relapsed/refractory setting for treatment of large B‐cell lymphoma (LBCL), chimeric antigen receptor T‐cell (CAR‐T) therapy has emerged as an effective treatment modality. Patients often have aggressive disease that requires prompt treatment in the form of bridging therapy (BT) for disease stabilisation while CAR‐T cells are manufactured. Patients (n = 75) undergoing CAR‐T therapy infusion for LBCL at our institution were identified. A total of 52 (69·3%) received BT and 23 (30·7%) received no BT (NBT). BT modalities included systemic BT (SBT) in 28 patients, radiation BT (RBT) in 14, and high‐dose steroid BT (HDS) in 10. There was no difference in incidence of cytokine release syndrome or immune effector cell‐associated neurotoxicity syndrome between BT and NBT (P = 0·18 and P = 0·53 respectively). Prolonged cytopenias at Day 180 were more common in BT than NBT (50% vs. 13·3%, P = 0·04). The SBT and RBT subgroups had more cytopenias at Day 180 compared to the HDS and NBT subgroups (58·3% and 57·1% vs. 20% and 13·3% respectively, P = 0·04). Disease response at last follow‐up, progression‐free survival and overall survival were similar between BT, NBT, and BT subgroups. In summary, BT can be safely considered in patients undergoing CAR‐T therapy. However, those undergoing BT with SBT or RBT are at higher risk of prolonged cytopenias after CAR‐T therapy. [ABSTRACT FROM AUTHOR]

Background: Human immunodeficiency virus (HIV) infection increases the risk of thrombotic microangiopathy (TMA), but TMA in the setting of HIV infection is not well characterized. The experience with TMA in the setting of HIV infection at the University of Maryland Medical Center was reviewed. Study design and methods: Patients undergoing therapeutic plasma exchange (TPE) for TMA from January 1, 2000 through December 31, 2012 were reviewed. Those with known HIV‐positive and ‐negative status were compared. Results: Among 102 patients with known HIV status, 28 (27%) were HIV‐positive, including 3 with previously undiagnosed HIV. HIV‐positive patients had a median viral load of 89 500 copies/mL (range, 0‐>750 000 copies/mL) and a median CD4 count of 58 cells/µL (range, 2‐410 cells/µL). Compared to HIV‐negative patients, HIV‐positive patients more frequently presented with concurrent infections (60.7% vs. 23.7%; P = .0007), had a trend toward lower median platelet counts (3000/µL vs. 15 000/µL; P = .07) and more frequently had platelet counts less than 10 000/mcL (P = .02). Nevertheless, number of TPE procedures required for remission, remission rate, mortality, and relapse incidence were similar in HIV‐positive and HIV‐negative patients. Conclusions: The incidence described herein of HIV infection among TMA patients is the highest reported outside of South Africa. More severe thrombocytopenia in HIV‐positive patients may reflect TMA in the setting of preexisting HIV‐associated thrombocytopenia. HIV should be considered in patients with TMA, and TMA should be considered in HIV‐positive patients with severe thrombocytopenia. [ABSTRACT FROM AUTHOR]

Haemoglobin concentration (Hb) was 50 g/l, white blood cell (WBC) count 3.8 × 10 SP 9 sp /l, neutrophil count 2.0 × 10 SP 9 sp /l and platelet count 164 × 10 SP 9 sp /l. Ferritin was 6 g/l and transferrin saturation 6%. Total serum bilirubin was 22.2 mol/l. Creatinine was normal. The absolute reticulocyte count was 205 × 10 SP 9 sp /l, total serum bilirubin 54.7 mol/l (direct, 3.4 mol/l) and lactate dehydrogenase 3533 iu/l. Creatinine was now 330 mol/l. His blood film showed normal red cell morphology. [Extracted from the article]