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Hepatosplenic T Cell Lymphoma
Introduction:
- It is a rare, systemic, mature T cell malignancy most often characterized by spleen, liver and bone marrow involvement and aggressive clinical course.
Epidemiology:
- Common in men
- Median age 35 years
- Accounts for <1% of all lymphomas
Etiology:
- Chronic immune stimulation in a setting of immunosuppression
Clinical Features:
- Hepatosplenomegaly
- Thrombocytopenia
- Anemia
- Sometimes- can present with HLH
Investigations:
- Liver biopsy:
- Lymphomatous cells are monotonous and medium sized. Nucleus contains loosely condensed chromatin and small inconspicuous nucleoli. Cytoplasm is pale
- Marked sinusoidal infiltration with sparing of portal triad
- Immunophenotyping
- Positive – CD3, CD2, CD7, Cytotoxic granule associated protein, TIA1
- Negative - CD4, CD8, CD5, perforin
- Variable: CD56
- Hemogram: Thrombocytopenia, Anemia, Leucocytosis
- Molecular studies-
- Rearrangement of TCR genes
- Activating mutations of JAK/STAT pathway (STAT5b, STAT3) and chromatin modifying genes (SETD2, INO80, ARID1B)
- Cytogenetics
- Isochromosome 7q
- Some times trisomy 8
- Loss of sex chromosomes
Prognosis:
- Aggressive
- Occasional survivors reported
- Median survival- 16 months
- Poor prognostic factors:
- Male gender
- Failure to achieve CR
- History of immunocompromise
- Absence of gene rearrangement in TCR
Pretreatment Work-up:
- History
- B-Symptoms
- Examination
- LN:
- Spleen:
- Full skin exam:
- WHO P. S.
- BSA
- IHC
- BMA and Bx
- CT (CAP)/ PET
- Stage
- Hemoglobin
- TLC, DLC
- Platelet count
- LFT- Bili- T/D SGPT: SGOT: Albumin: Globulin:
- Creatinine
- Electrolytes: Na: K: Ca: Mg: PO4:
- Uric acid:
- LDH
- HIV:
- HBsAg:
- HCV:
- HLH workup
- UPT
- HLA Typing
- ECHO(If anthracyclines planned)- LVEF- %
- Chemotherapy consent after informing about disease, prognosis, cost of therapy, side effects, hygiene, food and contraception
- Fertility preservation
- PICC line insertion and Chest X ray after line insertion
- Tumor board meeting and decision
- Attach supportive care drug sheet
- Inform primary care physician
Treatment Plan:
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