A case of hyperhemolysis syndrome in sickle cell disease and concomitant COVID-19.

BACKGROUND: Hyperhemolysis syndrome (HHS) is an uncommon transfusion reaction described in several hematologic disorders, including sickle cell disease (SCD). HHS is characterized by a decline in hemoglobin (Hb) values below pre-transfusion levels following transfusion of red blood cells (RBCs), coupled with laboratory markers consistent with hemolysis. The proposed pathophysiologic mechanisms underlying HHS include increased phosphatidylserine expression, macrophage activation, and complement dysregulation. Many pathophysiologic mechanisms thought to contribute to HHS have been similarly described in cases of severe COVID-19. CASE REPORT: A 28-year-old male with a history of HbSS presented with shortness of breath, right-sided chest pain, and a two-day history of fever. Polymerase chain reaction (PCR) detected SARS-CoV-2 infection with the omicron variant. The patient required an RBC transfusion (pre-transfusion hemoglobin [Hb]5.8 g/dL) with an immediate post-transfusion Hb of 6.3 g/dL. However, Hb rapidly declined to 1.7 g/dL, and lactate dehydrogenase (LDH) rose to 8701 u/L. The absolute reticulocyte count of 538 × 109/L correspondingly fell to 29 × 109/L. Despite additional RBC transfusions and initiation of immunosuppressive therapy, he expired on Day 9(D9). CONCLUSION: Given the similarities in their proposed pathophysiology, patients with SCD and concomitant SARS-CoV-2 infection may be predisposed to developing HHS.

Utilization of emicizumab in acquired hemophilia A: A case report.

BACKGROUND: Acquired Hemophilia A (AHA) is a rare autoimmune disorder associated with the development of autoantibodies against factor VIII (FVIII). Although obtaining hemostatic control through the use of recombinant factor VIIa, activated prothrombin complex concentrate and recombinant porcine FVIII are cornerstones in the clinical management of AHA, these therapies have several disadvantages, including a higher risk for the development of thromboembolic events, unpredictable efficacy and short half-lives. While emicizumab has been FDA licensed for use in bleeding prophylaxis for patients with Congenital Hemophilia A (CHA) with and without inhibitors, it has not been approved for use in AHA, with only a few reports describing its use in this context. CASE REPORT: We report our experience with the use of emicizumab in an 83-year old male with AHA, complicated by the onset of atrial fibrillation following admission, drug-induced thrombocytopenia, infectious complications, and the identification of a low-grade lymphoproliferative disorder, in which emicizumab prophylaxis was used for bleeding prophylaxis in the context of persistently elevated inhibitor titers without evidence of thrombotic events or thrombotic microangiopathy.

Effect of TPE vs medical management on patient outcomes in the setting of hypertriglyceridemia-induced acute pancreatitis with severely elevated triglycerides.

OBJECTIVES: Hypertriglyceridemia-induced acute pancreatitis (HTG-AP) accounts for 1 to 10% of pancreatitis cases, and is associated with a more severe clinical course. Therapeutic plasma exchange (TPE) is a potential treatment option for quickly lowering plasma triglycerides (TG). Current ASFA guidelines define HTG-AP as a Category III disorder, indicating the role of apheresis is not firmly established. Here, we examine clinical data regarding its effectiveness on morbidity and mortality in patients with HTG-AP presenting with severely elevated plasma triglycerides (>4000 mg/dl). METHODS: We retrospectively examined clinical data and outcomes from 67 consecutive episodes of HTG-AP over a 5-year period in which either medical management alone or medical management plus adjunct TPE was employed to reduce plasma triglycerides. RESULTS: 16/67 admissions involved TPE, initiated at a mean of 0.7 days from the time of presentation, while 51 received medical management alone. After only one TPE procedure, the mean TG values decreased from 4103 to 1045 mg/dl (a reduction of 74.7%), and those receiving TPE reached plasma TG < 1000 mg/dl 0.99 days faster than the medical group. One patient in the TPE group died. However, when excluding patients with hospital courses complicated by multiple organ dysfunction, there was no significant difference in mortality or hospital length of stay (LOS) between the groups. CONCLUSIONS: In uncomplicated cases of HTG-AP with an absence of multiorgan dysfunction, there is no significant benefit to either mortality or LOS when adding adjunct TPE to medical management, even when patients present with severely elevated levels of TG.