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Difficulty: HardHematology and Oncology

A 71-year-old woman is brought to the emergency department by her daughter due to a 3-day history of progressively worsening headache, dizziness, and blurry vision. The patient also reports having a severe nosebleed this morning that lasted for 30 minutes. Over the past 6 months, she has experienced significant fatigue and a 5-kg (11-lb) weight loss. She has no prior medical conditions and takes no medications. Vital signs are temperature 37.1°C (98.8°F), blood pressure 130/80 mm Hg, pulse 92/min, and respirations 16/min. Physical examination reveals conjunctival pallor and painless, rubbery cervical lymphadenopathy. Funduscopic examination demonstrates bilateral dilated, tortuous retinal veins with scattered flame-shaped hemorrhages. Laboratory studies show:

Hemoglobin: 8.2 g/dL
Platelets: 135,000/mm³
Leukocytes: 6,800/mm³
Blood urea nitrogen: 18 mg/dL
Serum creatinine: 1.0 mg/dL
Total protein: 11.2 g/dL
Albumin: 3.4 g/dL

Serum protein electrophoresis reveals a sharp localized band in the gamma region. Immunofixation electrophoresis confirms this is a monoclonal IgM kappa protein. Which of the following is the most appropriate next step in management for this patient?

  1. Therapeutic plasma exchangeAnswer
  2. B
    Bone marrow aspiration and biopsy
  3. C
    Phlebotomy
  4. D
    Administration of bortezomib and dexamethasone
  5. E
    Intravenous normal saline hydration

Answer

Therapeutic plasma exchange is the most appropriate next step to urgently resolve symptomatic hyperviscosity.
This patient's presentation of headache, dizziness, blurry vision, epistaxis, and 'sausage-link' (dilated and tortuous) retinal veins in the setting of a large monoclonal IgM spike is highly characteristic of hyperviscosity syndrome due to Waldenström macroglobulinemia (WM). IgM is a large pentameric immunoglobulin that significantly increases serum viscosity when present in high concentrations. This leads to impaired microcirculation, causing central nervous system symptoms and retinal venous engorgement. The most appropriate next step in management for symptomatic hyperviscosity is urgent therapeutic plasma exchange (plasmapheresis), which rapidly removes the IgM from the intravascular space and resolves the symptoms. Following stabilization, further diagnostic workup (e.g., bone marrow biopsy) and systemic chemotherapy can be pursued.

Step-by-Step Solution

1
Analyze the clinical presentation and laboratory findings.
The patient has headache, dizziness, vision changes, mucosal bleeding, retinal venous engorgement, and a large monoclonal IgM spike.
To establish the primary pathophysiology causing the patient's acute symptoms.
2
Diagnose the underlying condition and its acute complication.
The diagnosis is hyperviscosity syndrome secondary to Waldenström macroglobulinemia.
IgM is a large pentameric molecule; high levels significantly increase blood viscosity, leading to impaired microcirculation.
3
Determine the most appropriate immediate management.
Select therapeutic plasma exchange (plasmapheresis).
Mechanical removal of the IgM pentamers via plasmapheresis is required to rapidly reverse the potentially life-threatening neurologic and ocular complications of hyperviscosity.

Key Concept

Symptomatic hyperviscosity syndrome in Waldenström macroglobulinemia (IgM monoclonal gammopathy) is an oncologic emergency that requires immediate treatment with therapeutic plasma exchange.
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Topics

Hematology and Oncology

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