Department of Hematology, All India Institute of Medical Science, New Delhi, India
Case Report
PNH in a Child with Ringed Sideroblast-Diagnostic Riddle
Author(s): Richa Juneja*, Gurpreet Sagoo, Priyanka Mishra and Renu Saxena
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired stem cell disorder presenting either
with hemolytic or thrombotic event or co exists in patients with aplastic anemia and Myelodysplastic
syndromes (MDS). These PNH clones have been shown to have prognostic and therapeutic implications.
Flow cytometric analysis of GPI-linked proteins is the method of choice for diagnosis and it has replaced
the acidified-serum lysis test (Hams test) and sucrose lysis test being quantitative and a more sensitive
method. Occurrence of this PNH clones in pediatric patients is rare (10%) with few studies being
reported. It can have varied clinical and haematological manifestations and should be considered in
children especially in the setting of bone marrow failure causing cytopenia. We hereby present a rare
case of child having hemolytic manifestation of PNH with cytopen.. View more»