Langerhan’s cell histiocytoses

on 7.2.09 with 0 comments

  • Classified into different syndromes by clinicoanatomical patterns

  • Letterer-Siwe syndrome

    • AKA acute disseminated Langerhan's cell histiocytosis

    • Malignant, with systemic infiltrates

    • Seen in kids < 3 YOA and may be present at birth (something similar happens in adults)

    • F is followed by a diffuse maculopapular eczematous or pruritic rash with subsequent enlargement of the spleen, liver, and nodes throughout the body

    • Cystic lesions may appear in the skull, pelvis, and long bones

    • Cytopenias are frequent and are from marrow involvement

    • Death usually from intercurrent infections and progressive anemia

  • Unifocal Langerhans cell histiocytosis

    • AKA eosinophilic granulomas

    • Solitary, expanding, erosive accumulation of histiocytes with a variable admixture of eosinophils, lymphocytes, plasma cells, and neutrophils

    • Necrosis and multinucleated histiocytes may be present

    • Usually in medullary cavity of bones (skull, ribs, and femur most often)

    • Benign lesion in kids and adults, usually males; lesions can be asymptomatic or painful, and pathological fractures can occur

    • Sometimes spontaneous fibrosis and healing occur within a few years

  • Multifocal Langerhans cell histiocytosis

    • More disabling, onset before 5 YOA

    • Patients have F, a diffuse, scaly skin eruption (especially on scalp and in the ear canals), and frequent bouts of otitis media, mastoiditis, URI, and gingival inflammation; mild lymphadenopathy, hepatomegaly, and splenomegaly due to histiocytic infiltrates may be present

    • About 50% have DI

    • Orbital granulomas may produce exophthalmos (calvarial bone defects, DI, and exophthalmos = Hand-Schuller-Christian triad)

    • Good prognosis…50% have spontaneous remission and chemo is curative in the remainder

Category: Medical Subject Notes , Pathology Notes



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