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10/11/2014
1
Dr Stefan Dojcinov
University Hospital of Wales, Cardiff
Edinburgh Haematopathology Tutorial 2013 Case 7
Case 7
Male, 30
Splenomegaly and pancytopenia.
Splenectomy specimen submitted.
Spleen 1.6 kg, homogeneous on cutting.
Case 7
20%
77% 3%
10/11/2014
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TCR beta TCR gamma
Case 7 (MD2737) TCRB Tube A
Case 7 (MD2737) TCRB Tube B
Case 7 (MD2737) TCRB Tube C
TCRG Tube B Polyclonal Control
Case 7 (MD2737) TCRG Tube B
Case 7 – Additional History
Long standing history of Crohn’s disease since adolescence
12 months before presentation a flare up treated with azathioprine and infliximab
Case 7
Hepatosplenic T-cell Lymphoma
Azathioprine / Infliximab induced
Hepatosplenic T-cell Lymphoma (HSTCL)
Rare (<3% NHL)
Young adults (35-45), M>F
Long term immunosuppression
Transplantation
Azathioprine / Infliximab for Crohn’s
AWLP ©
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HSTCL Infliximab and Azathioprine Mackey et al. JPGN 2007; 44:265–267
• 8 Patients (12-31)
• Crohn’s (1UC)
• Infliximab, azathiprine, prednisolone
• As little as 1 infusion
• Devloped lymphoma between 7 months and 4 years after treatment
• 6/8 died
Hepatosplenic T-cell Lymphoma Presentation
Hepatosplenomegaly
No lymphadenopathy
No leukaemic component
Cytopenias
100% marrow involvement
AWLP ©
CD7
CD5
CD4
CD8
Granzyme B
TIA1 AWLP ©
TCR beta AWLP © TCR gamma
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Diagnostic Antibodies
• Gamma / Delta
Human Pan ”TCR gamma delta”
Clone 5A6.E91; Thermo Scientific, IL
• Gamma
TCR 1153 clone g3.20; Thermo Scientific, IL
Not developed for paraffin, but they would work
• Beta
– TCR1151, clone 8A3; Pierce Endogen, IL
HSTCL Diagnosis
Historically on splenectomy
Bone marrow diagnosis supported by flow cytometry immunophenotyping is becoming a standard
However, frequently missed in the marrow
CD7
TIA1 AWLP ©
Differential Diagnosis
Expansion of gamma/delta T-cells in SLE
NK/T nasal type
– EBV positivity detected in HSTL
– Absence of angiocentricity
– Strong TCR delta chain
– Evidence of a clonal TCR gene rearrangement
T-ALL
HSTCL - Treatment and Prognosis
No standardised protocol
– CHOP
– Vincristine
– Purine analogues
– Allogeneic transplantation
Very poor prognosis
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