Langerhans cell histiocytosis

Summary of Langerhans cell histiocytosis
Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans cells, dendritic cells of the skin. Clinically, its manifestations range from isolated bone lesions to multisystem disease. 

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Pathology

Hematological system

Anemias
Heme synthesis disorders
Coagulation disorders
Platelet disorders
Mixed platelet and coagulation disorders
Thrombosis syndromes (hypercoagulability)
Lymphomas
Leukemias
Leukemoid reaction
Dysplastic and proliferative disorders
Plasma cell dyscrasias
Hematological system pathology review

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Langerhans cell histiocytosis

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High Yield Notes
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Langerhans cell histiocytosis

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, CD207 (langerin), and S100 are the immunohistochemical markers that are indicative of Langerhans cell histiocytosis.

Questions

USMLE® Step 1 style questions USMLE

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USMLE® Step 2 style questions USMLE

2 questions
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A 7-year-old boy is brought to the clinic by his mother because of severe left leg pain. He localizes the pain to the middle of his left thigh and rates it as a 9 on a 10-point scale with no radiation. He does karate and his family recently got a cat. He denies weight loss, night sweats, or recent trauma. Temperature is 37.3°C (99.2°F), blood pressure is 127/81 mm Hg, pulse is 76/min, respirations are 14/min. X-ray shows a lytic lesion in the mid-shaft of the left femur. Biopsy of the lesion is performed, and microscopic pathology shows numerous cells with clear nuclei and "coffee-bean" shaped nuclei, as well as areas of eosinophils. Which of the following is the most likely diagnosis?

External References