Lymphoplasmacytic lymphoma and Waldenström Macroglobulinemia, A Decade After the Discovery of MYD88.

There has been remarkable progress over the past 80 years since Jan Waldenstrom first described patients with a hyperviscosity syndrome related to IgM paraprotein in 1944. The definition of Waldenstrom macroglobulinemia (WM) has evolved from a clinical syndrome to a distinct clinicopathologic entity with characteristic morphology, immunophenotype and molecular features. The landmark discovery of MYD88 mutation among most WM cases in 2012 marked the dawning of an era of molecular genomic exploration that led to a paradigm shift in clinical practice.

An emergency medicine review: Multiple myeloma and its complications.

Introduction: Multiple myeloma (MM) and its complications carry a high rate of morbidity and mortality.

Objective: This review evaluates MM and its complications, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence.

Discussion: MM is the second most common hematologic cancer and associated with monoclonal plasma cell proliferation.

Redefining Calciphylaxis as a Uniquely Bone Forming Subcutaneous C5b-9-Mediated Microvascular Injury Syndrome Associated With Localized Subcutaneous and Systemic Complement Pathway Activation.

Background: Microvascular thrombosis is key to the pathogenesis of calciphylaxis. C5b-9-mediated microvascular injury reflective of complement pathway activation could be a key pathophysiologic event.

Methods: We conducted a retrospective multicenter study of 24 patients who have had biopsy-supported calciphylaxis from the 2010-2022 data base from Emory where C5b-9 immunohistochemistry (IHC) had not been conducted and the 2019-2023 data base from Cornell where C5b-9 IHC was done as part of the routine calciphylaxis work up.

Hyperviscosity Syndrome in Immunoglobulin G4 (IgG4)-Related Disease and the Use of Therapeutic Plasma Exchange: A Case Report.

Immunoglobulin G4 (IgG4)-related disease is a rare multi-system immune-mediated inflammatory condition. Pathologically, it is associated with an increase in plasma IgG4 levels and tissue infiltration of IgG4-secreting plasma cells of any organ. Clinical features vary, but it usually presents with the diffuse enlargement and dysfunction of the affected organ.

Cryoglobulinemia Associated With Multiple Myeloma in a Dog Presenting With Epistaxis and Skin Lesions.

A 10-year-old female neutered Labrador Retriever presented with epistaxis, discoloration and crusting of the nose and a necrotic lesion on the lip. Bloodwork revealed pancytopenia, azotemia, hypoalbuminemia and hyperglobulinemia. Aggregates of amorphous basophilic material were seen in a room-temperature blood smear which were not present in the sample after warming to 37°C, and grossly a cryoprecipitate was noted in the patient's serum at 4°C.

Pathophysiology and Treatments of Complications of Waldenström's Macroglobulinemia.

Waldenstrom's macroglobulinemia (WM) or lymphoplasmacytic lymphoma is a B-cell malignancy characterized by lymphoplasmacytic cells in the bone marrow that secrete high amounts of immunoglobulin (Ig) M. The large pentameric structure of IgM leads to a variety of unique complications in WM, such as hyperviscosity syndrome, cryoglobulinemia and sensory neuropathy. Furthermore, malignant cells can infiltrate the central nervous system and lead to a variety of neurological complications, also known as Bing Neel Syndrome.

Successful treatment of a CLL associated IgM hyper-viscosity syndrome: A rare case.

In the context of chronic lymphocytic leukemia (CLL), Hyperviscosity Syndrome (HVS) typically arises from hyperleukocytosis, although it infrequently stems from IgM hyperparaproteinemia. We present a distinctive case of HVS induced by IgM hyperparaproteinemia in a patient experiencing relapsed CLL, marked by bulky disease and cytopenias upon progression. The patient exhibited new symptoms, including headache, dizziness, and confusion.

Optical coherence tomography angiography features in Waldenström macroglobulinemia patients without Hyperviscosity syndrome: A pilot prospective study.

Purpose: To evaluate the retinal vessel density (VD) with optical coherence tomography angiography (OCTA) in asymptomatic patients affected by Waldenström macroglobulinemia (WM) without hyperviscosity syndrome (HVS) and to highlight the presence of microvascular damage in theese clinically asymptomatic WD patients.

Design: Prospective study.

Methods: A total of 43 eyes from 43 WM patients (24 females, 19 males, mean age 55.