An unusual lupus like malar rash pattern with hepatosplenomegaly as a presentation of gamma delta t cell lymphoma

Ala’a Abdullah Hussein Bani-Mustafa; Ahmad Yousef Mustafa Magableh; Hisham Subhi Hussin bawa'neh; Zaid Nu’man Abdel Latif alrosan

doi:10.18535/ijsrm/v11i01.mp04

Abstract

About 12% of all lymphoid tumors worldwide are peripheral t cell lymphomas, which are uncommon subtypes of non-hodgkin lymphoma. they have a poor prognosis and are more common in populations and countries in asia. T cell lymphomas with gamma delta t cell receptors expressed in them are very rare (less than 1% of lymphoid neoplasms) and extremely aggressive; they originate from gamma delta t cells; a small subset of peripheral t cells with direct antigen recognition capability acting at the interface between innate and adaptive immunity .

Lymphoid tissue, skin, gastrointestinal tract, and the red pulp of the spleen are where gamma delta t lymphocytes tend to cocentrate, representing only 1-5% of the total circulating lymphocytes, but accounting for up to 30% of the entire t cell population.

In contrast to alpha beta t cells, gamma delta t lymphocytes develop from cd4/cd8 thymic precursors in the bone marrow and usually lack the major histocompatibility complex restriction.

These lymphocytes conduct as cytotoxic cells similar to natural killer cells in their activity as early effectors of innate, non-specific immune response and are capable of t cell receptor rearrangement as well as phagocytosis.

Hepatosplenic t cell lymphoma and primary cutanous gamma delta cell lymphoma are recognized as two different gamma delta t cell lymphoma entities.

A well characterized extranodal lymphoma, hepatosplenic t cell lymphoma has a disguised onset, secondary to intrasinusoidal infiltrationn of the spleen, liver, and bone marrow, has a rapidly progressive course, is poorly responsive to chemotherapy.

A panniculitis-like clinical picture with prominent epidermal involvement can be a presentation of primary cutaneous gamma delta t cell lymphoma.

In this case study , the authors will discuss a case of 19 year old male patient present with a lupus like malar rash and hepatosplenomegaly and who had been diagnosed with gamma delta t cell lymphoma.

Keywords

Gamma Delta T Cell LymphomaHepatosplenic T Cell LymphomaPrimary Cutanous

References

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Foppoli, Marco, and Andrés JM Ferreri. "Gamma‐delta t‐cell lymphomas." European journal of haematology 94.3 (2015): 206-218.‏Google Scholar ↗
Tripodo C, Iannitto E, Florena AM, Pucillo CE, Piccaluga PP, Franco V, Pileri SA. Gamma-delta T-cell lymphomas. Nat Rev Clin Oncol 2009; 6: 707– 17Google Scholar ↗
Iannitto E, Tripodo C. How I diagnose and treat splenic lymphomas. Blood 2011; 117: 2585– 95Google Scholar ↗
Wilhelm M, Meyer P, Batram C, Tony HP, Dummer R, Nestle F, Burg G, Wilms K. Gamma/delta receptor-expressing T-cell clones from a cutaneous T-cell lymphoma suppress hematopoiesis. Ann Hematol 1992; 65: 111– 5.Google Scholar ↗
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Belhadj K, Reyes F, Farcet JP, et al. Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. Blood 2003; 102: 4261– 9Google Scholar ↗
Sallah S, Smith SV, Lony LC, Woodard P, Schmitz JL, Folds JD. Gamma/delta T-cell hepatosplenic lymphoma: review of the literature, diagnosis by flow cytometry and concomitant autoimmune hemolytic anemia. Ann Hematol 1997; 74: 139– 42Google Scholar ↗
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Morton LM ,Wang SS ,Devesa SS , et al. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001.Blood ,2006, vol. 1071(pg. 265-276)Google Scholar ↗
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https://www.sciencedirect.com/science/article/abs/pii/S1040842811002320 , last accessed : 30 October 2022Google Scholar ↗
Cooke CB, Krenacs L, Stetler-Stevenson M, Greiner TC, Raffeld M, Kingma DW, Abruzzo L, Frantz C, Kaviani M, Jaffe ES. Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin. Blood 1996; 88: 4265– 74Google Scholar ↗
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Kotlyar DS, Osterman MT, Diamond RH, Porter D, Blonski WC, Wasik M, Sampat S, Mendizabal M, Lin MV, Lichtenstein GR. A systematic review of factors that contribute to hepatosplenic T-cell lymphoma in patients with inflammatory bowel disease. Clin Gastroenterol Hepatol 2011; 9: 36– 41.e1Google Scholar ↗
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Swerdlow S, McColl K, Rong Y, Lam M, Gupta A, Distelhorst CW. Apoptosis inhibition by Bcl-2 gives way to autophagy in glucocorticoid-treated lymphocytes. Autophagy 2008; 4: 612– 20.Google Scholar ↗
Weidmann E. Hepatosplenic T cell lymphoma. A review on 45 cases since the first report describing the disease as a distinct lymphoma entity in 1990. Leukemia 2000; 14: 991– 7Google Scholar ↗
Petit B, Leroy K, Kanavaros P, Boulland ML, Druet-Cabanac M, Haioun C, Bordessoule D, Gaulard P. Expression of p53 protein in T- and natural killer-cell lymphomas is associated with some clinicopathologic entities but rarely related to p53 mutations. Hum Pathol 2001; 32: 196– 204Google Scholar ↗
Sayers TJ, Brooks AD, Ward JM, Hoshino T, Bere WE, Wiegand GW, Kelly JM, Smyth MJ. The restricted expression of granzyme M in human lymphocytes. J Immunol 2001; 166: 765– 7Google Scholar ↗

[refR-1] Tripodo, Claudio, et al. "Gamma-delta T-cell lymphomas." Nature Reviews Clinical Oncology 6.12 (2009): 707-717.‏Google Scholar ↗

[refR-2] gamma delta t cell lymphoma , available at https://www.medscape.org/viewarticle/573559 , last accessed 1-november-2022Google Scholar ↗

[refR-3] Foppoli, Marco, and Andrés JM Ferreri. "Gamma‐delta t‐cell lymphomas." European journal of haematology 94.3 (2015): 206-218.‏Google Scholar ↗

[refR-4] Tripodo C, Iannitto E, Florena AM, Pucillo CE, Piccaluga PP, Franco V, Pileri SA. Gamma-delta T-cell lymphomas. Nat Rev Clin Oncol 2009; 6: 707– 17Google Scholar ↗

[refR-5] Iannitto E, Tripodo C. How I diagnose and treat splenic lymphomas. Blood 2011; 117: 2585– 95Google Scholar ↗

[refR-6] Wilhelm M, Meyer P, Batram C, Tony HP, Dummer R, Nestle F, Burg G, Wilms K. Gamma/delta receptor-expressing T-cell clones from a cutaneous T-cell lymphoma suppress hematopoiesis. Ann Hematol 1992; 65: 111– 5.Google Scholar ↗

[refR-7] Vega F, Medeiros LJ, Bueso-Ramos C, Jones D, Lai R, Luthra R, Abruzzo LV. Hepatosplenic gamma/delta T-cell lymphoma in bone marrow. A sinusoidal neoplasm with blastic cytologic features. Am J Clin Pathol 2001; 116: 410– 9Google Scholar ↗

[refR-8] Belhadj K, Reyes F, Farcet JP, et al. Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. Blood 2003; 102: 4261– 9Google Scholar ↗

[refR-9] Sallah S, Smith SV, Lony LC, Woodard P, Schmitz JL, Folds JD. Gamma/delta T-cell hepatosplenic lymphoma: review of the literature, diagnosis by flow cytometry and concomitant autoimmune hemolytic anemia. Ann Hematol 1997; 74: 139– 42Google Scholar ↗

[refR-10] 10 Tripodo C, Iannitto E, Florena AM, Pucillo CE, Piccaluga PP, Franco V, Pileri SA. Gamma-delta T-cell lymphomas. Nat Rev Clin Oncol 2009; 6: 707– 17Google Scholar ↗

[refR-11] 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– 8Google Scholar ↗

[refR-12] Morton LM ,Wang SS ,Devesa SS , et al. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001.Blood ,2006, vol. 1071(pg. 265-276)Google Scholar ↗

[refR-13] gamma delta t cell lymphoma , available atGoogle Scholar ↗

[refR-14] https://www.sciencedirect.com/science/article/abs/pii/S1040842811002320 , last accessed : 30 October 2022Google Scholar ↗

[refR-15] Cooke CB, Krenacs L, Stetler-Stevenson M, Greiner TC, Raffeld M, Kingma DW, Abruzzo L, Frantz C, Kaviani M, Jaffe ES. Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin. Blood 1996; 88: 4265– 74Google Scholar ↗

[refR-16] Gaulard P, Belhadj K, Reyes F. Gammadelta T-cell lymphomas. Semin Hematol 2003; 40: 233– 43Google Scholar ↗

[refR-17] Kotlyar DS, Osterman MT, Diamond RH, Porter D, Blonski WC, Wasik M, Sampat S, Mendizabal M, Lin MV, Lichtenstein GR. A systematic review of factors that contribute to hepatosplenic T-cell lymphoma in patients with inflammatory bowel disease. Clin Gastroenterol Hepatol 2011; 9: 36– 41.e1Google Scholar ↗

[refR-18] Allory Y, Challine D, Haioun C, Copie-Bergman C, Delfau-Larue MH, Boucher E, Charlotte F, Fabre M, Michel M, Gaulard P. Bone marrow involvement in lymphomas with hemophagocytic syndrome at presentation: a clinicopathologic study of 11 patients in a Western institution. Am J Surg Pathol 2001; 25: 865– 74Google Scholar ↗

[refR-19] Swerdlow S, McColl K, Rong Y, Lam M, Gupta A, Distelhorst CW. Apoptosis inhibition by Bcl-2 gives way to autophagy in glucocorticoid-treated lymphocytes. Autophagy 2008; 4: 612– 20.Google Scholar ↗

[refR-20] Weidmann E. Hepatosplenic T cell lymphoma. A review on 45 cases since the first report describing the disease as a distinct lymphoma entity in 1990. Leukemia 2000; 14: 991– 7Google Scholar ↗

[refR-21] Petit B, Leroy K, Kanavaros P, Boulland ML, Druet-Cabanac M, Haioun C, Bordessoule D, Gaulard P. Expression of p53 protein in T- and natural killer-cell lymphomas is associated with some clinicopathologic entities but rarely related to p53 mutations. Hum Pathol 2001; 32: 196– 204Google Scholar ↗

[refR-22] Sayers TJ, Brooks AD, Ward JM, Hoshino T, Bere WE, Wiegand GW, Kelly JM, Smyth MJ. The restricted expression of granzyme M in human lymphocytes. J Immunol 2001; 166: 765– 7Google Scholar ↗