List 3 causes of a massive splenomegaly.

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College Answer

°     CML

°     Myelofibrosis

°     Chronic malaria

°     Kala Azar

Discussion

This question is strange for somebody who has never given much thought to the precise clinical definition of "massive". How big is massive? Extending beyond the umbilicus? The size of my head?

Its all a bit arbitrary. One 1989 paper takes "massive" to mean "drained weight in excess of 1kg",which most people would agree is pretty big. In spite of the obvious dififculty of assessing the dry drained weight of a spleen in vivo, this definition seems to be consistent across such sources of enlightenment as Medscape eMedicine and Wikipedia. A slightly more workable definition of "massive splenomegaly" comes from Eric Poulin, even though he used the word "severe" instead - in his view anything more than 20cm in any dimension was a real cause for concern, and therefore merited a strong superlative.

Anyway. Where did the college get this list of unusual differentials?

Well.

Turns out, truly humongous splenomegaly can only occur in a small selection of disease states:

  • Kala Azar (visceral leishmaniasis)
  • Chronic malaria (hyper-reactive malarial splenomegaly)
  • Chronic tuberculosis
  • Chronic schistosomiasis
  • Primary splenic lymphoma
  • Myelofibrosis
  • Chronic myeloid leukaemia (CML)
  • Thalassaemia major or intermedia
  • Gaucher's disease
  • Sarcoidosis
  • POEMS syndrome
  • Polycythemia vera
  • Amyloidosis
  • Waldenstrom's macroglobulinaemia

All the other causes of splenomegaly (which I though still gave rise to some pretty impressive spleens) - like EBV, portal hypertension, etc - all of these spleens are rather pedestrian by comparison.

The causes of massive splenomegaly are briefly discussed in this tropical disease oriented article.

References

References

Hoffbrand's Essential Haematolgy has a table with the causes of splenomegaly in chapter 10.

 

Johnson, H. A., and R. A. Deterling. "Massive splenomegaly." Surgery, gynecology & obstetrics 168.2 (1989): 131-137.

 

Poulin, E. C., J. Mamazza, and C. M. Schlachta. "Splenic artery embolization before laparoscopic splenectomy." Surgical endoscopy 12.6 (1998): 870-875.

 

Bedu-Addo, George, and Imelda Bates. "Causes of massive tropical splenomegaly in Ghana." The Lancet 360.9331 (2002): 449-454.