Uncommon Presentation of a Common Leukemia (Chronic Lymphocytic Leukemia): Case Report
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MEDICINE
Uncommon Presentation of a Common Leukemia (Chronic Lymphocytic Leukemia): Case Report Francesca Martini 1 & Nadia Cecconi 1 & Giulia Cervetti 1 & Sara Galimberti 1 & Elena Ciabatti 1 & Mario Petrini 1 Accepted: 6 April 2020 / Published online: 18 April 2020 # Springer Nature Switzerland AG 2020
Abstract The association of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with usually low level of serum IgM monoclonal paraprotein is well recognized. We describe a rare case of a patient who developed overt IgM-related hyperviscosity syndrome after treatment for a diagnosis of CLL that was unresponsive to immunochemotherapy and to targeted therapies. Hyperviscosity syndrome, related to CLL-associated IgM paraprotein, is a very rare finding. High levels (> 3 g/dl) of serum IgM are mostly found in lymphoplasmacytic lymphoma-Waldenstrom macroglobulinemia (LPL/WM). In this patient, LPL was ruled out and diagnostic findings were consistent with only one clonal B cell population: CLL. It could be interesting understanding if ibrutinib therapy triggered IgM increase. Keywords Chronic lymphocytic leukemia . IgM paraprotein . Hyperviscosity syndrome . Therapy refractoriness
Abbreviations CLL/SLL Chronic lymphocytic leukemia/small lymphocytic lymphoma LPL/WM Lymphoplasmacytic lymphoma-Waldenstrom macroglobulinemia PCR Polymerase chain reaction NGS Next-generation sequencing FISH Fluorescent in situ hybridization TPA Therapeutic plasma exchange BTK Bruton tyrosine kinase
Background IgM paraprotein is often associated with CLL [1–4]. IgM values are usually low, but we describe an intriguing case of a patient who developed overt IgM-related hyperviscosity syndrome after treatment for a diagnosis of CLL that is responsive neither to immunochemotherapy nor to targeted therapies. This article is part of the Topical Collection on Medicine * Francesca Martini [email protected] 1
Department of Clinical and Experimental Medicine, U.O. Hematology, University of Pisa, Pisa, Italy
Case Presentation In 2010 a 47-year-old woman was diagnosed of stage II-B (Rai-Binet) CLL with normal karyotype (on bone marrow sample). In 2011, because of evidence for active disease, she was treated with 6 courses of rituximab-fludarabinecyclofosfamide followed by alemtuzumab, which induced a complete clinical remission. In 2014, at first relapse, the patient was treated with six courses of rituximab-bendamustine, obtaining a partial response. During the follow-up after treatment (from 2014 to 2016), IgM values progressively increased from 53 to 6550 mg/dl. The patient denied symptoms of hyperviscosity. Serum immunofixation revealed IgM k monoclonal gammopathy. Bone marrow biopsy identified monoclonal lymphocytes and plasmacytoid lymphocytes (8–10%) CD20+, CD23+, CD5+, and cIgMk+. B cell clonality testing by immunoglobulin heavy chain PCR, NGS, and flow cytometry (on bone marrow samples) was consistent with one clonal B cell population, according to the same light chain expressed on the CLL clone and by IgM paraprotein. MYD 88 L265P
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