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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%

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Case 7 Case 7

CD20 CD3

CD3 CD4

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CD8 CD5

CD2 CD7

TIA1 Granzyme

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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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