Husein, Yousuf A.

Jooma, Rashid

Kayani, Naila

Siddiqui, Shahid

Soomro, Irshad Nabi

Suhail Muzaffar, Suhail Muzaffar

English

eCommons@AKU

eCommons@AKU Department of Pathology and Laboratory Medicine Medical College, Pakistan 5-1998 Primary spinal T-cell rich B-cell lymphoma: A case report Suhail Muzaffar Suhail Muzaffar Irshad Nabi Soomro Naila Kayani Shahid Siddiqui Yousuf A. Husein See next page for additional authors Follow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiol  Part of the Neurology Commons, Neurosurgery Commons, Pathology Commons, Radiology Commons, and the Surgery Commons Authors Suhail Muzaffar Suhail Muzaffar, Irshad Nabi Soomro, Naila Kayani, Shahid Siddiqui, Yousuf A. Husein, and Rashid Jooma Primary Spinal T-Cell Rich B-Cell Lymphoma: A Case ReportPages with reference to book, From 148 To 149 Suhail Muzaffar,Irshad Nabi Soomro,Naila Kayani,Shahid Siddiqui ( Departments of Pathology, The Aga Khan University,Karachi. ) Yousuf A. Husein ( Departments of Radiology, The Aga Khan University, Karachi. ) Rasheed Jooma ( Department of Neurosurgery, Jinnah Postgraduate Medical Centre, Karachi. ) IntroductionNon-Hodgkin’s lymphoma is a heterogenous group of lymphoid malignancies with marked vanabiity inmorphologic features, growth pattern, antigemc phenotype and biological behaviout T- cell rich B-celllymphoma (TCRBCL) is a newly recognised entity of this large heterogenous group of lymphoidneopiasms which closely mimics Hodgkin’s disease and peripheral T-cell lymphoma (PTCL). Most ofthe cases of TCRBCL have been reported in the lymph nodes, however, a small number of cases havebeen described in the extranodal sites including skin, soft tissue, common bile duct and central nervoussystem. Primary spinal TCRBCL with symptoms of spinal cordcompressionhasbeen described in theliterature.Case Report A 30 year old man presented with history of paraplegia of three months duration. X-ray of the spinerevealed collapsed upper thoracic vertebra and paraspinal mass, however, the pedicles were preserved.Magnetic resonance imaging (MRI) showed partial collapse of upper thoracic vertebra and peripheralextension of the lesion into the spinal canal. The intervertebral discs below and above were preserved(Figure 1).Decompression procedure was performed and histopathological examination of the biopsy materialrevealed a cellular neoplasm predominantly composed of mature T lymphocytes as revealed byimmunocytochemistry. Amidst a small population of atypical cells (10-15%) with large irregular andlobulated nuclei, a small number of plasma cells and neutrophils were seen. Some of these atypicalcells show large prominent nucleoli. At places there was prominent vascularity. Foci of necrosis werealso present. The differential diagnosis in this case included Hodgkin’s disease and peripheral T-celllymphoma (PTCL). Immunohistochemical studies were carried out using a panel of lymphoid maikersincluding leucocyte common antigen (LCA), pan-B marker (L-26), pan-T marker (UCHL-1) andimmunoglobulin light chains (Kappa and Lambda). Phenotypically the large atypical cells were Blymphoid cells (L-26 positive) which also revealed clonal proliferation i.e., lambda light chainrestriction (Figure 2).Clinical and radiological workup failed to reveal any other souite of primary disease. So on the basis ofclinical, morphological and immunohistochemical features a diagnosis of primary spinal T-cell rich B-cell lymphoma(TCRBCL) was made.Discussion T-cell rich B-cell lymphoma (TCRBCL) is a newly recognised, unusual diffuse non-Hodgkin’slymphoma that contain a minority of neoplastic B cells surrounded by a predominant population ofreactive T-cells. This entity was first recognised and presented in abstract form as ‘pseudo-peripheral Tcell lyniphoma1’. However, some workers preferred the tcnn B-cell lymphoma resembling T-celllymphoma2. Ramsay eta! were the first who coined the term T-cell rich B-cell lymphoma (TCRBCL)for this lesion and this has gained wide acceptance3. The criteria for the diagnosis are now well definedas cited by Jaffe and include: a) Mimic peripheral T-cell lymphoma (PTCL) morphologically andphenotypically; b) Majority of the cells are phenotypically normal T-cells; c) Minority of the cells aremonoclonal B-cells and d)Immunohistochemistry (especially on paraffin sections) and moleculargenetics are useful in diagnosis -both Ig gene and be 1-2 rearrangements4.However, the upper limit for the number of neoplastic B-cells and minimum T-cells content withreference to the entire cellular population in TCRBCL is still controversial. Some workers used anarbitrary figure of 50% as minimum T-cells population in TCRBCL5, whereas Jaffe and co-workersproposed that it should be greater than 75%. According to Rainsay and colleagues the T-cell contentshould be 90% or above whereas Baddoura and co-workers proposed that ‘until more meaningful limitscan be established infuture studies, the pereentage of T and B-cells are reported in the majority of casesin the literature and in this study, should be used for the diagnoses of TCRBCL; an upper limit of 25%for the neoplastic B-cell population and a lower limit of 75% for T- lymphocytes. However, thepercentage of T-cells required for diagnoses may be reduced in cases with a large number ofhistiocytes6. Immunohistochemical analysis particularly on paraffin sections is essential to establish thediagnosis of TCRBCL and in most cases these studies are sufficient to establish the mononclonal natureof the B-cells. The most characteristic iminunophenotypic features are L-26 and monotypiccytoplasmic Ig reactivity by neoplastic cells.The main differential diagnoses for TCRBCL are peripheral T-cell lymphoma (PTCL) and Hodgkin’sdisease, particularly the lymphocyte predominant type (LPHD). In the later the presence of L&H cells,lack of conspicuous vascularity, significant mixture of small B-cells and inability to demonstrate B-cellmonoclonality are important distinguishing features. PTCLs pose important diagnostic problems, asTCRBCLs are easily confused with this morphologically heterogenous group of non-Hodgkin’slymphoma (NHLs). Majority of the TCRBCLs are diffuse and may resemble one of the patternsdescribed for PTCLs, for example, lymphocytic infiltrate of mixed size, compartmentalisation bydelicate connective tissue septa and hypervasculanty. TCRBCL with prominent histiocytic componentmay resemble Lennert’s lymphoma. Cases of TCRBCL with T-zone distribution and sparing of benignfollicles have also been described. It is worth noting that a number of cases which have been placed inthe categoiy of PTCLs might have actually TCRBCLs. For a pathologist it is important to recognisethis newly emerged entity and distinguish it from Hodgkin’s disease and peripheral T cell lymphornabecause of different therapeutic modalities. With reference to the International Working Formulationthere are basically two school of thoughts for placing TCRBCLs into this clasification system. Onegroup feels that mixed population of morphologically atypical small and large Ivmphoid cells should betaken into account and the lesion should be placed in diffuse mixed cell (DMC) category. The othergroup argues that since only the large cell are neoplastic, these cases should be classified into either thediffuse large cell (DLC) or the diffuse large cell immunoblastic (DLCI) subtypes whereas in RevisedEuropean American Lymphoma (REAL) classification this entity has been placed in category of diffuselarge B cell lvmphoiua. Since DMC, DLC and DLCI lymphomas are treated similarly in mostinstitutions, so this issue is ofonly academic importance. The clinicaldataofthese lymphonias is limited.Majority of the cases are presented in adults in their sixties with a slight male predominance.To date approximately 95 to 100 cases of TCRBCL have been reported in the international literature,most of which are seen in the lymph nodes3,6,7. However, a handful of cases have been reported asprimary extranodal TCRBCL including skin6, soft tissue7, common bile duct8 and central nervoussystem9. Our case is probably the first case report of primary extranodal TCRBCL arising in the spine(Stage IE) and causing cord compression.References 1. Jaffe, ES. Pseudo-peripheral T-cell lymphomas. In Jaffe, ES. ed. Surgical pathology of lymph nodesand related organs. Philadelphia, WB Saunders, 1985, pp. 230-231.2. Mirchandani, I., Palutke, M., Tabaczka, P. et al. B-cell lymphomas morphologically resembling T-cell lymphomas. Cancer, 1985.56:1578-1583.3. Ramsay, A.D.,Smith, \\V.J and lsaacson, P.O. T-cell rich B-cell lymphoma. Am. J. Surg. Pathol.,1988;12:433-443.4. Jaffe, ES., T-cell rich B-cell lymphoma (TCRBCL). In Jaffe, ES. ed. Surgical pathology of lymphnodes and related organs. Philadelphia, W.B. Saunders, 1995, pp. 356-358.5. Ng, CS., Chan, J.K.C.. Hui, P,K. et al. Large B-cell lymphomas with a high contentofreactive I-lymphocytes. Hum. Pathol., 1989;20:1145.1 154.6. l3addoura, F.K.. Chan, \\V C., Masih, AS. etal. T. cell rich B- cell lymphoma: A clinicopathologicstudy of eight cases. Am. J. Clin. Pathol., 1995; 103:65-75.7. Macon, W.R., Williams, M E., Grcer, J.P. e al. T-cell rich B- cell lymphomas: Aclinicopathologicstudy of 19 cascs. Am. J. Surg. Pathol., 1992;16:351-363.8. Brouland, J.P., Molimard, J., Ncmcth, J. et al. Primary 1-cell rich B-cell lymphoma ofthecommonbileduct. Virchows. Arch. Pathol. Anat. Histopathol., 1993;423 :5 13-5 17.9. Okuyama, J., Fujit.a, T., Sato, K et al. Primary malignant T-ccll rich B-cell lymphoma of the centralnervous system: A case report. No Shinkei. Geka, 1995;23:1003-1006.

Primary spinal T-cell rich B-cell lymphoma: A case report

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Primary spinal T-cell rich B-cell lymphoma: A case report

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