Thursday, September 15, 2011

IN THE NEWS:- Myeloproliferative neoplasms and glomerular disease

A recent article referenced below in Kidney International Sept 2011 issue reveals a novel glomerular disease associated with myeloproliferative neoplasms( CML, polycythemia vera, essential thrombocytosis, mastocytosis, primary myelofibrosis and other) that usually become AML.
A description of 11 cases showed following characteristics:
1. mesangial scloerosis
2. hypecellularity
3. Segmental sclerosis
4. Chronic TMA findings
5. Intracapillary hematopoietic cell infiltration
6. No signs of immune complex mediated process by EM and IF

The term MPN related glomerulopathy is suggested.  This is interesting to note as clinically I have always wondered about the nephrotic syndrome that is associated with these cases of MPN. In the literature, there is information about the MPGN pattern of injury seen with such cases and perhaps this is just semantics but looking at the findings suggests a thrombotic microangiopathy and MPGN pattern of injury with novel findings mainly of the cell infiltration( which might have been there in prior cases published but not looked for ).
Morphologically, have to be differentiate from diabetic glomerular changes, FSGS, TMA, and immune complex MPGN

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