Cryoglobulinemic vasculitis and marginal zone lymphoma in HCV

Dr. Kearney presented an interesting case of an elderly gentleman with a history of PVD and COPD who presented with chronic progressive bilateral lower extremity necrotic ulcerations, found to have massive splenomegaly on exam in addition to his skin findings, ultimately diagnosed with cryoglobulinemic vasculitis and marginal zone lymphoma in the setting of hepatitis C.

Learning Points:

Etiologies of Leg Ulcers

Common: venous, arterial, and neuropathic

 Table comparing the three major types of leg ulcers.

Table comparing the three major types of leg ulcers.

Uncommon:

  • Infection (bacterial, fungal, spirochete, protozoal)
  • Vasculopathies (livedoid vasculopathy, Buerger’s disease)
  • Vasculitis (HSP, mixed cryo, PAN, GPA, SLE, rheumatoid vasculitis)
  • Vaso-occlusive disorders (calciphylaxis, cholesterol emboli, type I cryo, oxalosis)
  • Neoplastic conditions (SCC, BCC, cutaneous T and B cell lymphoma, Kaposi’s sarcoma)
  • Drug-induced (warfarin, heparin, hydroxyurea)
  • Hematologic diseases (hemoglobinopathies, thrombocytosis)
  • Pyoderma gangrenosum
  • Spider bite
  • Among others!

DDx of Massively Enlarged Spleen

  • CML
  • Myelofibrosis
  • Gaucher disease
  • Lymphoma, usually indolent, including hairy cell leukemia
  • Kala-azar (visceral leishmaniasis)
  • Hyperreactive malarial splenomegaly
  • Beta thalassemia major
  • AIDS with mycobacterium avium complex

Ultimately, our patient was diagnosed with splenic marginal zone lymphoma. Dr. Kearney presented evidence that treating with antivirals to eradicate HCV can lead to complete remission of the lymphoma. Most of the studies used older regimens of IFN and ribavirin; however, we may be able to extrapolate the data to newer direct anti-viral agents.