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Jaundice in Langerhan cell histiocytosis is a manifestation of liver disease; which may be attributed to hepatocellular
dysfunction or hemolysis. Cholestasis may result from lymph nodes obstructing the porta hepatis. This report describes a
case of type II histocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant
lymphadenopathy at the porta. A 2 year old boy with progressive abdominal distention, right ear discharge and seborrhoeic
dermatitis at since 6 months of age was studied. He also had a one month history of fever, jaundice and skin lesions on his scalp
for an uncertain duration. He had firm hepatosplenomegaly and shifting dullness. Laboratory findings revealed mild anemia.
Liver chemistries revealed AST of 119 U/l, ALT 50 U/l, ALP of 4045U/L and GGT of 924IU/l and a total serum bilirubin
of 10.0 mg/dl with direct fraction of 8.9 mg%. Bone marrow examination revealed mild plasmacytosis, dyserythropoiesis
consistent with hepatic disease, but no evidence of metastasis. Contrast enhanced tomography of the chest revealed multiple
centrilobular nodules in bilateral lung field diffusely exhibiting ground glass opacity, with cystic changes in the upper lobe.
Skeletal survey showed multiple osteolytic punched out lesions in the parietal areas of the skull bilaterally and right iliac
bones. The skin biopsy of the scalp was showed CD1a positive and S100 positive histiocytic cells. He completed chemotherapy
as per protocol and showed progressive improvement throughout his course. Although there is multisystem involvement in
LCH, health practitioners must be cognizant that LCH can present as cholestatic jaundice without significant porta hepatis
lymphadenopathy.
Biography
Rohit Kapoor has completed his MD (Postgraduation) in Pediatrics at the age of 26 years from Vardhman Mahavir Medical College and Safdarjang Hospital, New
Delhi, India. He is currently employed as a Medical Officer under the Government of India. He has completed his thesis during Post-graduation on "Retinopathy of
prematurity in infants with birth weight between 1500 grams to 1800 grams". He has cleared the USMLE examinations and wishes to specialize in combined Internal
Medicine and Pediatrics residency or in Pediatrics separately.
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