RESUMO
A 22-days-old male newborn baby presented with persistence of neonatal jaundice since birth. On clinical examination he had coarse facial features, a prominent forehead, enlarged tongue, icterus, hepatosplenomegaly, skeletal deformities and bilateral inguinal hernia. On investigation the peripheral smear revealed Alder-Reilly anomaly in the neutrophils suggesting mucopolysaccharidosis. Mucopolysaccharide excretion spot test of the urine was positive; and an assay for glycosaminoglycans in the urine was also high, which confirmed the clinical diagnosis of Hurler's syndrome. We present this rare case to highlight the association of Alder-Reilly anomaly and bilateral inguinal hernia in Hurler's syndrome even in neonates.
RESUMO
Plasma cell leukemia (PCL) is a rare type of plasma cell dyscrasia. It is diagnosed when circulating plasma cells (PC) are more than 20%. We present a case of PCL in a 62-year-old female. Peripheral smear revealed more than 80% atypical vacuolated plasma cells (Mott cells) almost mimicking Burkitt cells of Acute Lymphoid Leukemia-L3 (ALL-L3). Bone marrow aspirate revealed few mature myeloma cells for which a diagnosis of PCL was thought of. Serum electrophoresis showed a positive M-band and X-ray revealed lytic lesions over femur & pelvic bones. A final diagnosis of PCL was given.