Challenge your diagnostic skills

Question 2 of 3 – What is your next step?

Further investigations were performed to establish the cause of the recurrent TMA.

• Physical examination: Repeat physical exam revealed an enlarged subclavicular lymph node. Purpura, gross hematuria, and abdominal pain resolved after plasmapheresis. Hepatomegaly or splenomegaly were not detected. The patient did not present with neurologic deficits.
• Laboratory tests: Tests for hepatitis B and C virus, HIV infection, antinuclear bodies, and anti-phospholipid antibodies were negative.

Immunofixation electrophoresis detected monoclonal IgMk in the serum. The cryoprecipitate also contained polyclonal IgG and immunoglobulin light chains k and λ.

No anti-factor H antibodies or C3NF were detected.

LDH, platelets, and C3 levels returned to normal.

Cryoglobulinemia, C4 hypocomplementemia, and proteinuria persisted.

Immunofixation electrophoresis findings. (A) Monoclonal IgMκ in the serum. (B) Monoclonal band was not detected since complete remission.

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