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malignant histiocytosis; true histiocytic lymphoma; reticulosarcoma; histiocytic medullary reticulosis
Epidemiology:
1) nonates, children, adults
2) males affected more frequently than females
3) wide geographic occurrence
Pathology:
1) proliferation of atypical histiocytes
- acid phosphatase & nonspecific esterase positive
2) atypical histiocytes are pleomorphic; nucleoli are visible
3) hemophagocytosis by atypical histiocytes variable
4) mitotic figures may be seen
5) lymph node biopsy
a) focal or diffuse lymph node involvement
b) effacement of lymph node & capsular invasion
6) biopsy of other soft tissues; examination of surgical specimens (liver, spleen)
7) 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:
1) fever
2) weakness, malaise, weight loss
3) sweating
4) chest pain
5) back pain
6) rashes
7) lymphadenopathy, localized or general (most patients)
8) hepatosplenomegaly (common)
a) may present as asymptomatic splenomegaly
b) jaundice & ascites are later features
Laboratory:
1) complete blood count (CBC)
a) anemia present in most patients on presentation
b) thrombocytopenia (frequent), thrombocytosis (occasional)
c) leukopenia (frequent)
2) serum bilirubin may be increased
3) serum lactate dehydrogenase may b increased
4) bone marrow aspirate & biopsy may not be diagnostic
Differential diagnosis:
1) non Hodgkin's lymphoma
2) Hodgkin's disease
3) hemophagocytic histiocytic syndrome
Management:
1) untreated, a rapidly progressive & fatal disease
2) combination chemotherapy
a) cyclophosphamide, doxorubicin, vincristine & prednisone
b) chloromethane, vincristine, procarbazine & prednisone
c) etoposide & cytosine arabinoside
d) complete remission > 50%, but short-lived (months)
3) Bone marrow transplantation
4) most patients die within one year of onset, but some may live > 5 years.
Interactions
disease interactions
Specific
Letterer-Siwe syndrome
General
histiocytic neoplasm
histiocytosis
malignant neoplasm (cancer)
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: 845260 Free PMC Article