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Now presented with bleeding from nose and gums - 2 days and vomiting on/off 2 months.
O/E : pallor + petechiae on lower limbs, Massive spleen.
No active bleed at the moment.
CBC shows pancytopenia (plat 11). P/F shows polychromasia and NRBC.
Cytopenia's multifactorial : 1: marrow replacement, 2: splenic sequestration 3: Drug induced (antiepileptic)
Supportive Transfusion for now (hb>8 and Plat > 10000) .
Needs enzyme replacement therapy imiglucerase.
Transplant is an option.
Spleenectomy last resort (may be required if ERT not available)
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