Hereditary spherocytosis: experience of 145 cases.
145 patients were diagnosed to have hereditary spherocytosis (HS) over a period of 25 years. Jaundice (66.9%), fever (65.5%), weakness (44.8%), and abdominal pain (35.8%) were the commonest complaints. 94.5% had splenomegaly (JP-17 cm) and 71.7% had hepatomegaly (JP-6 cm). Spherocytes were detected in the peripheral smears of all patients at presentation on careful examination. 67 patients had been investigated elsewhere and spherocytes missed in 86.6%. Gall stones were seen in 20 of the 54 patients investigated. Family history suggestive of HS was available in only 16.6% of cases, whereas examination and investigations revealed HS in almost all families. Splenectomy was done in all symptomatic patients. In the 39 patients followed up for 1-9 years after splenectomy.
Mehta
J
J
JC Patel Department of Hematology, KEM Hospital, Bombay.
Harjai
K
K
Vasani
J
J
Banghar
P
P
Sanklecha
M
M
Singhal
S
S
Pathare
A
A
Tilve
G H
GH
Mehta
B C
BC
Patel
J C
JC
D000328:Adult; D000368:Aged; D002675:Child, Preschool; D003906:Developing Countries; D003937:Diagnosis, Differential; D005260:Female; D006452:Hemoglobinometry; D006801:Humans; D007194:India; D008297:Male; D008875:Middle Aged; D013103:Spherocytosis, Hereditary / Q000175:diagnosis / Q000235:genetics*; D013156:Splenectomy