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Year : 2018  |  Volume : 61  |  Issue : 2  |  Page : 292-293
Leukemic conversion of hepatosplenic T-cell lymphoma with pleomorphic morphology and an aggressive course


1 Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India

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Date of Web Publication20-Apr-2018
 

How to cite this article:
Sharma P, Nampoothiri RV, Sharma P, Naseem S, Malhotra P, Varma N. Leukemic conversion of hepatosplenic T-cell lymphoma with pleomorphic morphology and an aggressive course. Indian J Pathol Microbiol 2018;61:292-3

How to cite this URL:
Sharma P, Nampoothiri RV, Sharma P, Naseem S, Malhotra P, Varma N. Leukemic conversion of hepatosplenic T-cell lymphoma with pleomorphic morphology and an aggressive course. Indian J Pathol Microbiol [serial online] 2018 [cited 2020 Feb 21];61:292-3. Available from: http://www.ijpmonline.org/text.asp?2018/61/2/292/230555




A 24-year-old male presented with generalized weakness, fatigue, and dyspnea-on-exertion for 4-months. He was pale with massive splenomegaly and moderate hepatomegaly without lymphadenopathy. Investigations revealed pancytopenia: hemoglobin 72 g/L, total leukocyte count 3.8 × 109/L, and platelet count 39 × 109/L. Bone marrow aspirate revealed 48% abnormal, highly pleomorphic cells with indented to floret-shaped, lobulated nuclei, clumped chromatin, and moderate amount of basophilic cytoplasm with blebbing and fraying. Some showed cytoplasmic granules and fine vacuoles [Figure 1]. They were negative on cytochemical myeloperoxidase staining. The trephine biopsy showed intrasinusoidal infiltration by these cells. Morphological differential diagnoses included high-grade non-Hodgkin lymphoma and acute monoblastic leukemia.
Figure 1: Peripheral blood film showing several aggressive-looking tumor cells having pleomorphic morphology (Leishman, ×1000)

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Flow cytometrically [Figure 2], the cells expressed CD45, surface and cytoplasmic CD3, CD2, CD7, CD16, CD56, and TCR-γδ. They were negative for CD5, CD4, CD8, TCR-αβ, CD34, CD57, TdT, HLA-DR, and CD117. A diagnosis of hepatosplenic T-cell lymphoma (HSTCL), Ann Arbor stage 4 was rendered.
Figure 2: Flow cytometric scatter plots showing the gated cells (CD45 bright events) positive for CD3, CD2, CD7, TCRγδ, CD16, and CD56, and negative for CD20, CD4, CD8, CD5, CD1a, TCRαβ, CD34, HLA-DR, and CD117

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The patient was started on prephase prednisolone 60 mg/m 2/day. However, by day 3, he developed increasing shortness of breath and diffuse petechial rash. Hemoglobin fell to 56 g/L with total leukocyte count 68.9 × 109/L and platelet count 19 × 109/L. Peripheral smear showed 82% atypical cells with morphology similar that seen previously and flowcytometry reconfirmed the diagnosis of HSTCL, now in leukemic transformation. He died a week later.

HSTCL is difficult to treat and typically have poor outcomes with median survival <3 years.[1],[2],[3] Marrow infiltration occurs in nearly two-thirds of the patients at diagnosis.[2] Leukemic spills in peripheral blood are uncommon and can create diagnostic difficulties, as in our case, when acute leukemias and high-grade non-Hodgkin lymphomas (diffuse large B-cell or blastic mantle cell types, for instance) may be considered morphologically. The intrasinusoidal pattern may be subtle and missed unless a high degree of suspicion is maintained.[2] Bright CD45 expression and absent immaturity markers on flow cytometry as well as immunohistochemistry can help resolve such situations.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Mastovich S, Ratech H, Ware RE, Moore JO, Borowitz MJ. Hepatosplenic T-cell lymphoma: An unusual case of a gamma delta T-cell lymphoma with a blast-like terminal transformation. Hum Pathol 1994;25:102-8.  Back to cited text no. 1
    
2.
Das R, Sachdeva MU, Malhotra P, Das A, Ahluwalia J, Bal A, et al. Diagnostic difficulties of pure intrasinusoidal bone marrow infiltration of non-Hodgkin's lymphoma: A report of eight cases from India. Jpn J Clin Oncol 2011;41:1303-7.  Back to cited text no. 2
    
3.
Yabe M, Medeiros LJ, Tang G, Wang SA, Ahmed S, Nieto Y, et al. Prognostic factors of hepatosplenic T-cell lymphoma: Clinicopathologic study of 28 cases. Am J Surg Pathol 2016;40:676-88.  Back to cited text no. 3
    

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Correspondence Address:
Prashant Sharma
Department of Hematology, Level 5, Research Block A, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_495_17

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