malignant histiocytosis; true histiocytic lymphoma; reticulosarcoma; histiocytic medullary reticulosis
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Epidemiology
- nonates, children, adults
- males affected more frequently than females
- wide geographic occurrence
Pathology
- proliferation of atypical histiocytes
- acid phosphatase & nonspecific esterase positive
- atypical histiocytes are pleomorphic; nucleoli are visible
- hemophagocytosis by atypical histiocytes variable
- mitotic figures may be seen
- lymph node biopsy
- focal or diffuse lymph node involvement
- effacement of lymph node & capsular invasion
- biopsy of other soft tissues; examination of surgical specimens (liver, spleen)
- bone marrow aspirate &/or biopsy
- initially negative biopsies should be repeated as disease progresses with time
Genetics
- translocations involving chromosome 5q35 in some
Clinical manifestations
- fever
- weakness, malaise, weight loss
- sweating
- chest pain
- back pain
- rashes
- lymphadenopathy, localized or general (most patients)
- hepatosplenomegaly (common)
- may present as asymptomatic splenomegaly
- jaundice & ascites are later features
Laboratory
- complete blood count (CBC)
- anemia present in most patients on presentation
- thrombocytopenia (frequent), thrombocytosis (occasional)
- leukopenia (frequent)
- serum bilirubin may be increased
- serum lactate dehydrogenase may b increased
- bone marrow aspirate & biopsy may not be diagnostic
Differential diagnosis
- non Hodgkin's lymphoma
- Hodgkin's disease
- hemophagocytic histiocytic syndrome
Management
- untreated, a rapidly progressive & fatal disease
- combination chemotherapy
- cyclophosphamide, doxorubicin, vincristine & prednisone
- chloromethane, vincristine, procarbazine & prednisone
- etoposide & cytosine arabinoside
- complete remission > 50%, but short-lived (months)
- Bone marrow transplantation
- most patients die within one year of onset, but some may live > 5 years.
More general terms
More specific terms
References
- ↑ Williams Hematology, 5th ed, Beutler et al (eds), McGraw Hill, NY, 1995, pg 890
- ↑ Wikipedia: Malignant histiocytosis https://en.wikipedia.org/wiki/Malignant_histiocytosis
- ↑ Macgillivray JB, Duthie JS Malignant histiocytosis (histiocytic medullary reticulosis) with spindle cell differentiation and tumour formation. J Clin Pathol. 1977 Feb;30(2):120-5. PMID: https://www.ncbi.nlm.nih.gov/pubmed/845260 Free PMC Article