We describe a case of a patient with multiple myeloma with initial presentation simulating a bleeding disorder. Detailed coagulation work-up showed hypodysfibrinogenaemia along with a platelet function defect consistent with acquired Bernard -Soulier syndrome (BSS). Multiple plasma exchanges led to significant improvement in his bleeding symptoms. To the best of our knowledge, this is the first described case of simultaneous presentation of hypodysfibrinogenaemia and BSS secondary to multiple myeloma. Blood Coagul Fibrinolysis 33:130-133
Introduction:Neurological symptoms may occur in patients with chronic lymphocytic leukemia (CLL) through a variety of etiologies. However, autoimmune disorders involving the nervous system, such as Guillain-Barre syndrome (GBS), have only rarely been reported in patients with CLL, despite the well-established association between CLL and other autoimmune phenomena. Previous reports demonstrate no consistent approach to management of these cases.Case Report: Here we report a case of severe GBS associated with newly diagnosed CLL. The patient did not respond to initial therapy with intravenous immunoglobulin, plasmapheresis, and corticosteroids, but demonstrated a rapid neurological and hematological improvement following cyclophosphamide infusion.
Conclusion:Here, for the first time, we describe the successful treatment of CLL-associated GBS using cyclophosphamide.
Chronic eosinophilic leukaemia, not otherwise specified (CEL, NOS), is a diagnosis of exclusion made in cases in which there is clonal eosinophilia but an absence of genetic aberrations that define other disease subtypes. There is a need for further characterization of these cases in order to inform risk stratification and management. The importance of <i>JAK2</i> mutations in myeloproliferative neoplasms (MPN) as a whole is well established, although their role specifically in eosinophilic disorders is less clear, with only a minority of cases demonstrating <i>JAK2</i> abnormalities. Here, we report 2 cases with an exon 13 insertion-deletion (indel) mutation in <i>JAK2:</i> one with CEL-NOS and the second with an unspecified eosinophilic disorder. <i>JAK2</i> indels were not detected in a screen of suspected MPN cases (<i>n</i> = 592) without eosinophilia that tested negative for common MPN driver mutations. Our findings thus provide further evidence for a specific association between this rare mutation and clonal eosinophilic disorders.
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