Soumya Pandey

Optia® continuous mononuclear collection (CMNC) system is a safe and efficient system for hematopoietic progenitor cells-apheresis (HPC-a) collection and yields a lower product hematocrit (HCT%) than the COBE® spectra system: A retrospective study.

We evaluated the Optia® continuous mononuclear collection (CMNC) system for hematopoietic progenitor cell‐apheresis (HPC‐A) collection (Terumo BCT, Lakewood, CO) compared to the COBE® Spectra (Terumo BCT, Lakewood, CO), including both large volume leukapheresis (LVL) and non‐LVL collections.

Prolonged prothrombin time correlates with serum monoclonal protein concentration in patients with plasma cell dyscrasia

Abnormal screening coagulation tests are frequently observed in asymptomatic patients with multiple myeloma and other plasma cell neoplasms.

Heparin-induced thrombocytopenia: a cautionary tale

A 32-year-old man with a history of multiple sclerosis was hospitalized for a significant flare, and heparin for deep vein thrombosis (DVT) prophylaxis was started. He did not respond to medical management alone, requiring intubation for ventilatory support; so a right internal jugular apheresis catheter was placed on Hospital Day 12 (HD12) and therapeutic plasma exchange (TPE) was initiated. TPE was performed again on HD14 and the patient tolerated both procedures without difficulty. On HD15 his platelet count decreased to 120 3 10/L (353 3 10 on HD12, 256 3 10 on HD14). Heparin was discontinued due to clinical suspicion of heparin-induced thrombocytopenia (HIT), but HIT ELISA testing was negative (OD, 0.262; <0.4 is negative). TPE on HD16 was aborted halfway through due to clotting. A clot (approx. 17 3 3 3 0.2 in.) was noted in the plasma bag (see figure) and microscopic examination was consistent with a fibrin clot. Venous duplex examination revealed DVT of the right internal jugular vein and obstruction of the right subclavian vein. Repeat HIT ELISA test was performed and the result was positive (OD, 3.666; >50% inhibition in the presence of heparin). Serotonin release assay was not performed. The patient did well on fondaparinux anticoagulation. Since TPE is known to remove antibodies, the patient’s TPE procedures may have contributed to the initial negative ELISA test results. This case emphasizes the fact that a negative ELISA for HIT may be misleading; if clinical suspicion for HIT is high, heparin therapy should be discontinued, appropriate alternative anticoagulation should be initiated, and a repeat or functional assay should be considered.

Lingual Leishmaniasis Complicating Visceral Disease

Leishmania species are obligate intracellular parasites transmitted by various types of female sand flies. The clinical syndrome that results depends on a number of factors including the Leishmania species and immune response of the host. Here, we report successful treatment of lingual leishmaniasis complicating visceral disease in an immunocompetent patient.

Complement Regulatory Genetic Mutations in the Setting of Autoimmune Thrombotic Thrombocytopenic Purpura: A Case Series

Objective To explore the benefits of adding eculizumab for the treatment of refractory autoimmune thrombotic thrombocytopenic purpura (iTTP) with complement dysregulation. Patients and Methods From January 1, 2014, through July 1, 2017, we identified patients with iTTP defined by ADAMTS13 (disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) levels less than 5% and the presence of ADAMTS13 inhibitor. Patients who progressed after receiving standard of care management for iTTP were subjected to a comprehensive evaluation to look for evidence of complement activation. Herein, we share our single-institute experience regarding the clinical course and treatment algorithm for 3 patients with refractory iTTP. Results All the patients had clinical deterioration despite treatment with plasma exchange, corticosteroids, rituximab, and vincristine, which prompted us to look for evidence of complement activation and associated genetic mutations. Complement-related genetic aberrations were present in all 3 patients, who had had different degrees of complement activation. The first 2 patients did not benefit from eculizumab when treatment was started before complete clearance of inhibitors to ADAMTS13. However, they had durable remissions when eculizumab was introduced after clearance of ADAMTS13 inhibitors. The third patient started eculizumab therapy after inhibitor levels were undetectable. Conclusion We found eculizumab therapy to be effective in all 3 patients. However, its efficacy was prominent only after clearance of antibodies against ADAMTS13 via therapeutic plasma exchange.

Morphologic and Immunohistochemical Evaluation of Plasma Cell Neoplasms

Plasma cell myeloma (PCM) is a bone marrow-based neoplasm, the diagnosis of which is straightforward in most cases. The presence of marked cytologic pleomorphism and extramedullary involvement, for example, can make the diagnosis of myeloma a difficult one in some instances. This chapter will provide a comprehensive review of the morphologic features of PCM; however, our emphasis will be on those variant histologic, cytologic, and immunophenotypic features that provide important diagnostic clues or that have the potential for misinterpretation leading to possible misdiagnosis. Because it is predominantly a neoplasm of the bone marrow, our discussion will focus primarily on bone marrow findings in PCM.

Rituximab and intermediate‐purity plasma‐derived factor VIII concentrate (Koate®) as adjuncts to therapeutic plasma exchange for thrombotic thrombocytopenic purpura in patients with an ADAMTS13 inhibitor

Thrombotic thrombocytopenic purpura (TTP) results from a congenital or acquired deficiency of the von Willebrand factor (vWF)‐cleaving protease ADAMTS13. The disease can be fatal and hence treatment should be initiated promptly. Therapeutic plasma exchange (TPE) remains the standard treatment along with adjunct therapies including steroids and immunosuppressive drugs. Addition of rituximab to TPE has been shown to be beneficial in refractory/relapsing TTP; however, TPE results in removal of rituximab from the circulation requiring more frequent dosing of rituximab to achieve a favorable outcome. The intermediate‐purity plasma‐derived Factor VIII concentrate (FVIII) Koate® contains the highest amount of ADAMTS13 activity yet reported and has been used successfully in treating congenital TTP. Here we report our experience with addition of this FVIII concentrate to rituximab, corticosteroids and TPE in three TTP patients with an ADAMTS13 inhibitor to permit withholding TPE for 48 h after rituximab infusion. J. Clin. Apheresis 30:50–54, 2015.