Case 50 part 1 -summary

Thanks for all of the contributions. The marrow showed erythrophagocytosis. This can be a sign of autoimmune haemolytic anaemia. In particular it can be seen in paroxysmal cold haemoglobinuria. We were slightly mean and didn’t show the rest of the marrow, which did not show any sign of haemophagocytosis (cells other than red cells being phagocytosed by macropahges) and that there was an excess of small mature lymphocytes. In addition the red cells looked agglutinated on the smear, which is often a sign of cold haemolytic anaemia. However in this case the DAT was IgG and the patient was clinically not behaving like someone with cold haemolysis. I think it was just artifact on the slide.

 

When evaluating someone with possible haemolytic anaemia the following investigations are required:

  • FBC – anaemia, macrocytosis, reticulocytosis
  • Blood film – spherocytes, agglutination, any signs of lymphoproliferative disease
  • LFTs – unconjugated hyperbilirubinaemia
  • Haptoglobin – low
  • LDH – elevated
  • DAT – positive in autoimmune

 

The most common causes of warm haemolytic anaemia are lymphoproliferative disorders, drugs, autoimmune disease and idiopathic. Our patient had lymphadenopathy and some weight loss. The marrow aspirate (as well as erythrophagocytosis) showed an excess of small mature lymphocytes, which by flow cytometry confirmed a typical CLL/SLL immunophenotype (CD19+, CD5+, FMC7-, CD23+, CD79a+(wk), sIg+(wk)). Immunohistochemistry showed a rather extensive nodular infiltrate with CLL/SLL. Transformation of CLL/SLL to a high grade lymphoma was considered, so lymph node biopsy was carried out which was consistent with CLL/SLL.

Paroxysmal cold haemoglobinuria is a rare condition whereby haemolysis occurs after an antibody attaches to the red blood cells in the cold, only to induce complement-mediated haemolysis when the patient is re-warmed. This commonly presents in younger children following a viral infection whereby they become rapidly anaemic and suffer from haemoglobinuria. Syphilis can also cause this as well as lymphoproliferative disorders. Testing for the biphasic antibody is via the Donath-Landsteiner test. This was negative in our patient.

 

In conclusion our patient had CLL/SLL which had provoked warm autoimmune haemolysis causing erythrophagocytosis. He was commenced on steroids initially which resulted in a rapid clinical improvement.

 

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