PREGNANCY COMPLICATED WITH HEREDITARY SPHEROCYTOSIS – LESSONS LEARNT FROM A CASE AND REVIEW OF LITERATURE.

Hereditary spherocytosis (HS) also known as Minkowski-Chauffard syndrome is a genetically determined disorder of the red blood cell membrane cytoskeleton complex causing Hemolytic anemia. There is a wide spectrum of clinical presentation ranging from mild anemia to severe anemia with splenomegaly and jaundice. Increased Mean Corpuscular Hemoglobin Concentration (MCHC), spherocytes in peripheral blood smear and increased osmotic fragility favour the diagnosis of HS.No causal treatment is yet available for this disease. Mild disease warrants no treatment. Folic acid, supportive treatment and regular annual check up usually suffice for moderate disease while frequent hematological supervision and splenectomy is prudent for severe disease.Pregnancy complicates the management of HS and very limited data is available in the literature regarding this. Case Presentation:-Authors hereby report a case of 24-year-old 36-weeksgestational age primigravida withintrauterine fetal demise. Workup revealed patient to be suffering from Hereditary Spherocytosis that resulted in hemolysis ultimately causing fetal demise. Pregnancy was terminated and patient was subsequently referred to the Department of Surgery for splenectomy. Conclusion:- Hereditary Spherocytosis, one of the commonest congenital hemolytic anemia may get decompensated during pregnancy. Due to paucity of data in literature regarding management of HS during pregnancy, it is hard to formulate guidelines for indications of splenectomy during pregnancy. However, authors recommend Splenectomy preferably in second trimester in any pregnant women with HS who is experiencing hemolysis or having symptoms, signs and complications thereof. Obstetricians should exercise high index of suspicion to diagnose this not so uncommon entity as early as pregnancy is diagnosed to yield better outcomes.