Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 2 years old male child was admitted in Dhaka Shishu Hospital with one month history of fever, swelling and pain in joints of right leg. Hematological and microbiological investigation revealed normal except radiological findings when there were destructive lesions of affected bones and thought to be Langerhan's cell histiocytosis but did not respond to treatment. Subsequently, he was transfer to Pediatric Hematology and Oncology, Bangabandhu Sheikh Mujib Medical University (BSMMU) for proper management. On examination, the boy was mildly pale, severe bone pain, no organomegaly but few occipital group of lymphnode were palpable. He had extensive red tender swelling over the hands and legs. Hematological values showed high WBC, low platelet count and lymphoblasts. Biochemical values were high serum LDH and serum uric acid. Skeletal survey showed diffuse osteolytic lesion and osteoporosis with evidence of transverse metaphyseal radiolucent bands (leukemic line), lamellar periosteal reactions and cortical erosions widespread throughout the skeleton with subperiosteal new bone formation. Radioisotop of skeleton showed increase uptake at the site of lesions. Bone marrow aspiration findings were suggestive of ALL-L1 and cytochemistry of aspirate showed PAS positive and Sudan black negative. Immunophenotype confirmed as pre-B ALL, Then, protocol based induction had given for 4 weeks. Thereafter, a short course of intensification followed by maintenance therapy had started with significant improvement of physical, hematological and radiological findings. So, an awareness of varied clinical and radiological manifestations of childhood ALL in bone marrow and skeletal system are needed in order to establish a correct diagnosis when the presenting signs and symptoms are enigmatic. It is also important to keep in mind the possibility of extensive skeletal involvement in ALL, especially when a child present with pain, swelling of limb with walking difficulties. The curability of ALL in contrast to metastatic malignancies makes accurate diagnosis of paramount importance.
 ...
PMID:Extensive skeletal lesions in childhood acute lymphoblastic leukemia. 1918 58