Birbeck granules are seen in the cytoplasm of
Correct Answer: Langerhans cells
Description: Langerhans cell histiocytosis (LCH)*Langerhans cell is a dendritic cell which is an antigen presenting cell*LCH is a clonal histiocytic disorder*Birbeck granules are present in the cytoplasm of Langerhans cell.Characteristic features of LCHPresence of Birbeck granules - Electron microscopy ( Tennis racquet appearance)Presence of enzymes like 1. a- Naphthyl Acetate Esterase 2. a- Naphthyl Butyrate Esterase 3. Acid phosphatasePresence of markers S-100, CD1a positivity HLA- DR expressionThree clinicopathological entities1.Acute disseminated LCH2.Multifocal LCH3.Unifocal LCH *Earlier Histiocytosis x term was used. This includes-Letterer-Siwe disease (generalized disease)-Hand - Schuller- Christian disease-Eosinophilic granuloma (localized lesion) However now the term LCH is preferred.Acute disseminated LCH ( Letterer- Siwe disease) - Affects children < 2 yrs-Seborrhoeic type of skin rash on front & back of trunk and scalp-Moderate hepatomegaly-Few palpable lymph nodes-Pulmonary lesions- uncommon-Bone- Lytic lesions (> 80%)-Moderate degree fever-Otitis media & mastoiditis may be seen-CNS involvement due to contiguous spread-Hematologic findings: Pancytopenia-Bone marrow: Infiltration by histiocytesMultifocal LCH - Multiple lesion involving multiple systems-Prognosis better than disseminated LCH-Fever & rash-Involvement of posterior pituitary- Diabetes insipidus-Typical Hand- Schuller Christian disease= Triad of exopthalmus+ diabetes insipidus+ calvarial defectUnifocal LCH*Focal lytic lesion in bone*Excellent prognosis(Ref: Robbins 8/e p631, 7/e p701, 702)
Category:
Pathology
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now