Pathology · Hematopathology

A 66-year-old man reports fatigue, blurring of vision, and epistaxis. Examination shows fundus venous engorgement with sausage-like segmentation of retinal veins. Investigations reveal Hb 8.4 g/dL, ESR 4 mm/hour, IgM level 42 g/L, and bone marrow infiltration by lymphoplasmacytic cells. The visual symptoms are explained primarily by:

  • A Direct deposition of monoclonal light chains in the vitreous
  • B Occlusion of central retinal artery by cryoglobulin aggregates
  • C Markedly increased plasma viscosity from pentamer formation of the paraprotein
  • D Optic nerve compression by orbital plasmacytoma
Correct answer: C. Markedly increased plasma viscosity from pentamer formation of the paraprotein

Explanation

Waldenstrom macroglobulinemia features a monoclonal IgM paraprotein secreted by lymphoplasmacytic lymphoma cells in the marrow. Because IgM circulates as a large pentamer confined to the intravascular space, it disproportionately raises plasma viscosity, producing hyperviscosity syndrome with retinal vein sausage-linking, mucosal bleeding, and neurologic symptoms. Light chain deposition and cryoglobulins are complications of other plasma cell disorders, and orbital plasmacytoma is not part of this entity.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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