1. CASE OF THE MONTH Dr Narender Tejwani Consultant ( Hematopathologist)
Department of Laboratory Services
Rajiv Gandhi Cancer Hospital & Research Centre
Rohini, New Delhi

2. Clinical History
56 year old male presented to hematology OPD with complaints for weakness, fatigue and dragging abdominal pain for last 6 months
Evaluated outside and found to have raised leukocyte count
History of blood transfusion present
No history of bleeding or fever

3. Clinical Examination Vitals: Stable,
Pallor present, No icterus, No bleeding, No fever
Systemic Examination:
Respiratory system & CVS: Normal
No lymphadenopathy
Massive splenomegaly reaching upto umbilicus on per abdominal examination

4. INVESTIGATIONS

5. Complete Blood Counts Hemoglobin: 8.4 gm%
Platelet Counts: Per Cumm
Total Leukocyte Counts: Per Cumm

6. Peripheral Smear
Red cells: Microcytic Hypochromic with many target cells
WBC: Show presence of Neutrophillia, Monocytosis and lymphocytosis with 9% intermediate to large atypical cells with scant cytoplasm and relatively clumped chromatin. Few show blastoid morphology. No dysplasia seen.
Platelets reduced on smear

7. 1 2
Intermediate sized cell with scant cytoplasm and clumped chromatin
Intermediate sized cell with scant cytoplasm and opened up chromatin
Resembling blast with prominent nucleoli 3

8. Bone Marrow Aspirate
Cellular Marrow with showing all hemopoietic cells adequately
No dysplasia seen
24% atypical cells with morphology similar as described in smear

9. Differential Diagnosis
Contradictory findings
Non hodgkin lymphoma
Acute Leukemia
Blast crisis in MDS/MPN
Blast crisis in MPN
No lymphadenopathy
Long history
No dysplasia
Only 6 month history, normal megakaryocyte morphology
FLOWCYTOMETRY WAS ADVISED FOR FURTHER WORKUP

10. Flowcytometry
SINGLETS SELECTED
DEBRIS GATED OUT

11. FLOWCYTOMETRY CD45 VS Side Scatter Findings
The cells were gated in bright CD45 region of lymphocytes

12. FLOWCYTOMETRY CD3 Positive Cells Findings
B cell analysis was unremarkable
T cells were gated on SSC Vs CD3 plot
All further analysis are described on CD3 positive T cells

13. FLOWCYTOMETRY CD2 Vs CD5 Findings
An abnormal T cell population was identified which was negative or CD5 (Red in color)
Normal T cell Population is labeled green

14. FLOWCYTOMETRY CD4 Vs CD8 Findings
The abnormal population was negative for CD4 and CD8
Note the normal T cells in green showing isolated CD4 or CD8 positivity

15. FLOWCYTOMETRY CD5 Vs CD7 Findings
The abnormal cells were bright CD7 positive
Normal cells are dual CD5 and CD7 positive

16. FINAL DIAGNOSIS An abnormal T cell population was identified
Positive: CD3, CD2, CD7
Negative: CD5, CD4, CD8
TCR receptor by flow was sent to referral lab and show Gamma Delta Restriction (Plot not available)
A final diagnosis of “HEPATOSPLEINC T CELL LYMPHOMA-GAMMA DELTA TYPE” was made (In view of massive spleen and no lymph node)

17. Hepatoslpenic T Cell Lymphoma

18. Introduction
Peripheral T cell lymphomas constitute ~15% of all NHL in adults
Hepatosplenic T cell lymphoma is a rare subtype of this group characterized by primary involvement of liver and spleen
This is a group of aggressive neoplasm that were initially called as hepatopslenic gamma delta T cell lymphoma
Later term gamma delta was removed as some of the cases show Alpha Beta TCR

19. Epidemiology & Pathogenesis
Rare neoplasm with less than 100 cases in literature
Median age is 35 years with slight male predominance
Pathogenesis is largely unknown. Some associations are:
20% cases are immunosuppressed
Majority have isochromosome 7q

20. Clinical History Splenomegaly: 100% cases Hepatomegaly: ~50% cases
B Symptoms: ~70% cases
Lymphadenopathy: <10% cases
Thus the patient presents with lymphomatous involvement of spleen without any evidence of lymph node involvement
This is very peculiar presentation of this group amongst all T cell lymphomas

21. Morphology
The group is characterized by monotonous intermediate sized lymphoid cells with moderate cytoplasm, round or slightly irregular nuclei, relatively opened up chromatin and indistinct nuclei.

22. Immunophenotype Positive markers: CD2, CD3, CD7 and CD16.
Negative markers: CD5, CD4 and CD8
Rare: CD4+ or CD8+ or Dual positive variants described
TCR restriction: Gamma Delta type but rare Alpha Beta variant are also described

23. Diagnosis
Characteristic Clinical: Massive splenomegaly without lymphadenopathy
Immunophenotypic: CD3, CD2, CD7 positivity and Negativity for CD4, CD8 and CD5
TCR Restriction: Expression of Gamma Delta TCT strongly supports the case however not must
Isochromosome 7q: Not required for diagnosis but strongly supports the case

24. Differential Diagnosis
Differentiating Feature
PTCL NOS
ALCL Systemic Type
Angioimmunoblastic T NHL
Lymphoblastic Lymphoma
T-LGL
Aggressive NK cell leukemia
Splenic Marginal Zone Lymphoma, Hairy Cell Leukemia
Generalized lymph node +
Pleomorphic cell, LN +
Shows immaturity markers
Subtle course, CD8+, αß+
CD3 negative
B Cell markers positive

25. Thanks