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NON-NEOPLASTIC DISEASES OF HEMATOPOETIC ORGANS Pathology. Jan Żeromski 200 10 /20 11. PATHOLOGY OF THYMUS. Developmental defects – a plasia (Di George anomal y) , Nezelof syndrome
- Benign-mostly of medullaryepithelialcells
caused by infection with microbes activating B cells.
due to infection with microbes activating T cells.
accumulation of histiocytes in lymphatic sinusoids. Occurs in LNs draining cancers (of breast and/or stomach).
Typical epithelioid cell tubercles with Langhans` giant cells. HE, 100x
No caseation, though there are scattered areas of necrosis. Note epithelioid cell zone with several Langhans` giant cells.
Center: necrotic area surrounded by wide zone of epithelioid cells with a few Langhans` giant cells. At the upper boundary of the central wedgeshaped area of necrosis there is some nuclear debris. Diagnosis serologically confirmed. HE, 65x
Epithelioid cell nodules without caseation; much fibrosis and abundant giant cells. Center: an “asteroid body”. HE, 100x
Hist. - diffuse hyperplasia and an increase of immunoblasts.
Sympt. - skin rash, fever, hepatosplenomegaly, painful joints.
Hist. - widened pale-stained T-zone areas with foaming vacuolated macrophages with fat and/or melanin droplets.
HIV-relatedlymphadenopathy (verylargelymphoidfollicleswithgiant germinal centers, laterfragmentation and atrophy)
Lymphadenopathyinrheumatoidarthritis (enlargement of lymphoidfollicles, lowproliferativeactivity of germinal centers, accumulation of plasmacellsbetweenfollicles, infiltration of neutrophilsinsinuses
Lymphadenopathyininfectiousmononucleosis(in paracorticalareaproliferation of immunoblasts – mostly B cells, with high mitoticactivity
Two major grosstypes :
single localized– large tumor inmediastinum, non-specificsymptoms: fever, anemia, hypo-proteinemia, polyclonalhipergammapathy;goodprognosis
Multifocal– general lymphadenopathy,highfever, weightloss, skin changes, hemolysis, poli- ormono-hypergammapathy, possibleprogress to lymphoma; poorprognosis
Probableetiology: infectionwithherpesvirus HHV-8
Histology – twotypes:
vascular hyaline- atrophy of germinal centersinlymphoidfollicles, numerousvenulessurrounded by hyalinemasses(angiofollicular lymphnode hyperplasia)
(predominantin single localisedtype)
plasma cellular– interfollicularspacesfilledwithplasmacells
Septicemia arising from liver abscesses due to echinococcosis.Spleen weight 610g.
Hyperplasia of reticulum cells in red pulp. Note presence of large pulp cells separated from their normal connection. Leucocyte infiltration, erythrophagocytosis. HE, 520x
Note active proliferation of reticulum cells, deployment of littoral cells, erythrophagocytosis. HE, 520x
Suppression of myeloid stem cells
Neoplastic occupation of bone marrow
Suppression of granulocyte precursors
Vit. B12 or folate deficiency
Inreased utilization-in infections
Exposure to drugs
Proliferation of CD8+ LGL in bone marrowLEUKOPENIA-USUALLY GRANULOCYTOPENIA (BELOW 1000/c.mm)
Monocytes, macrophages, reticulum cells, histiocytes, endothelia, dendritic cells.
Unifocal: eosinophilic granuloma
Multifocal:Hand – Schuller - Christian dis.
also - fever, skin eruptions, hepatospleno-megaly
Systemic: acute disseminated (Letterer - Siwe disease
Proliferation of histiocytes filled up with glucocerebroside - lack of glucocerebrosidase, an enzyme needed to hydrolize glycolipids from senescent blood cells
Mutation on chromosome 1 (q21-q31)
Splenohepatomegaly (giant spleen up to 10 kg), erosion of bones, anemia, leucopenia, various neuropathies
Infiltration of organs with Gaucher cells rich in glucocerebrosides stored in lysosomes