Case 3 – summary

Issues raised during this case:
* Comments written on a new probable CLL film: most people tell the GP that the patient probably has CLL and suggest GPs should discuss patients with nodes or splenomegaly. Most people suggest repeating the FBC in 4-6 months. Some people wondered how ethical it was to effectively make a ‘serious diagnosis’ without seeing the patient.
*Prognostic scores: many people pointed to the p53 mutation as a prognostic marker, and in particular the resistance it confers to Fludarabine. There was less mention, although it was raised, that p53 mutated patients are also likely to have disease resistant to rituximab, bendamustine and alkylating agents.
*Prognostic scores: other methods of prognostication raised included IgHV gene analysis, CD38 by flow, 11q deletions.
*Timing of such testing was briefly discussed. Guidelines suggest we shouldn’t check these markers outside of clinical trials and this was mentioned. Equally it was pointed out that in patients considered suitable for transplantation this information is useful in the early stages.
* Our patient developed an isolated anaemia. AIHA was the very reasonable first thought of most people but the reticulocytopenia made this less likely; the differentials we then established were pure red cell aplasia and heavy marrow infiltration with CLL leading to marrow failure. Other thoughts included AIHA with exhausted haematinics and drug reaction with a selective effect on erythropoiesis.
* In true haematology style we reached for our marrow needle and found that the marrow should a complete absence of erythroblasts, with no excess of lymphocytes and normal granulopoiesis and megakaryopoiesis. This fits with PRCA, an immune moderated (in CLL) destruction of erythroid precursors which is rare, but underrecognised, in CLL patients.Parvovirus b19, as a transient cause of red cell aplasia was mentioned and should be excluded.
* Management of PRCA was suggested; the focus was on immunosuppression with either ciclosporin, rituximab or steroids, used in varying combinations.

Useful papers for your consideration on these topics include:
http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2141.2008.07216.x/full
http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2141.2011.08707.x/full
http://www.bcshguidelines.com/documents/chronicLL_05052004.pdf

If you would like to suggest other useful papers please tweet and I will update this page.

Thanks for your participation. Join us for Case 4 next week!

About TeamHaem

Online education and discussion about all things haematological
This entry was posted in Anaemia, Chronic leukaemia and tagged , . Bookmark the permalink.

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