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histiocyte-rich B-cell lymphoma of spleen: A case report
APME-393; No. of Pages 3 apollo medicine xxx (2017) xxx–xxx
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/apme
Case Report
T-cell/histiocyte-rich B-cell lymphoma of spleen: A case report Srikanta Panda a, Roshni Tirkey b, Anshuman Sarangi c,* a
Associate Professor, P.G. Department of Surgery, S.C.B. Medical College and Hospital, Cuttack, Odisha, India Junior Resident, P.G. Department of Surgery, S.C.B. Medical College and Hospital, Cuttack, Odisha, India c Research Associate, Big Project, KIIT-TBI, KIIT University, Bhubaneswar, Odisha, India b
article info
abstract
Article history:
Splenomegaly is a frequent yet challenging condition that clinicians have to deal within
Received 26 October 2016
their daily clinical practice. Although the spleen is commonly involved in disseminated
Accepted 11 November 2016
lymphoma, primary lymphoma of the spleen is rare. A great majority of the reported cases
Available online xxx
are of B-cell lineage, with diffuse large B-cell lymphoma being the most common. A new variant of T-cell/histiocyte-rich B-cell was first described by Dogan et al. in 2003.1 The
Keywords:
combined morphologic, immunohistochemical, and molecular genetic data are essential for
Splenic lymphoma
a correct diagnosis of splenic T-cell-rich B-cell lymphoma. We report a classical case of T-
T-cell-rich B-cell lymphoma of
cell-rich B-cell lymphoma of the spleen in a 45-year-old male patient with clinical diagnosis
spleen
of splenomegaly. © 2017 Indraprastha Medical Corporation Ltd. All rights reserved.
Spleen Large B-cell lymphoma Splenomegaly
1.
Case report
A 45-year-old, Hindu male was presented with pain in the left upper abdomen that was spreading to the left shoulder since 6 months. On examination, a palpable left upper quadrant abdominal mass of 15 cm 18 cm was found in the supine and right lateral decubitus position with knees up and hips flexed. The mass extended from left the subcostal region up to the right iliac fossa slightly crossing the midline. Medial border and notch were palpable. On Ultrasound Sonography Test (USG) examination, a huge splenomegaly was confirmed (Figs. 1–4). Routine investigations were within normal limits. Vaccination against Pneumococcus, H-influenza type B, and
Meningococci type C were given 2 weeks prior to elective splenectomy. A left subcostal incision beginning two fingers breadth below the costal margin starting from the midline to the tip of the 11th costal cartilage was made. After operation, a huge spleen was isolated and sent for histopathologic examination to the Pathology Department, S.C.B. Medical College, Cuttack. Surgical pathology specimen received from splenectomy measured 23 cm 17 cm 11 cm with splenic notch. The specimen was grayish brown in color. Multiple sections were made from the cortex and the medulla. The histosections show splenic capsule with complete effacement of splenic architecture by small to intermediate to large, round to polygonal cells dispersed among the fibrocollagenous stroma
* Corresponding author. Tel.: +91 9861232063/9556078270. E-mail address: [email protected] (A. Sarangi). http://dx.doi.org/10.1016/j.apme.2016.11.009 0976-0016/© 2017 Indraprastha Medical Corporation Ltd. All rights reserved.
Please cite this article in press as: Panda S, et al. T-cell/histiocyte-rich B-cell lymphoma of spleen: A case report, Apollo Med. (2017), http:// dx.doi.org/10.1016/j.apme.2016.11.009
APME-393; No. of Pages 3
2
apollo medicine xxx (2017) xxx–xxx
Fig. 1 – Huge spleen after splenectomy.
Fig. 4 – Ki 67 positivity in less than 2% of the cells.
in vague follicular pattern. ImmunoHistoChemistry (IHC) for CDs shows that 80% of the cells are strongly positive. Cells are CD20 positive in large lymphocytes with vesicular nuclei constituting 10% of the cell population. Cells are also positive for bcl 6 and CD 79a but negative for CD 5, bcl-2, CD 10, CD 30, and CD 15. Ki-67 proliferation index was less than 2%, and in large cells it was 90%. With these findings, a diagnosis of T-
cell-rich B-cell lymphoma of spleen was given. Then the patient was advised an examination of the bone marrow, which did not reveal any infiltration by the lymphoma cells.
Fig. 2 – Haematoxylin and Eosin stain (HE) slide showing splenic lymphoma cells.
Fig. 3 – CD3 reactive proliferation of T-cells.
2.
Discussion
Incidental finding of an isolated splenomegaly during clinical assessment of patients evaluated for unrelated causes has become increasingly frequent because of the widespread use of imaging. A true diagnostic dilemma hides in distinguishing pathologic conditions primarily involving the spleen from those in which splenomegaly presents as an epiphenomenon of hepatic or systemic diseases.1,2 To qualify for primary splenic status, the lymphoma should be confined to the spleen and/or splenic hilar lymph nodes, although some authors also accept the presence of bone marrow involvement. Micronodular T-cell/histiocyte-rich B-cell (THRBCL) lymphoma of spleen was first reported and described by Dogan et al. in 2003.1 It is a type of Non-Hodgkin Lymphoma (NHL) characterized by a limited number of scattered large, atypical B-cells embedded in a background of abundant T-cells and frequently along with histiocytes. It accounts for less than 10% of all diffuse large B-cell lymphomas. It mainly affects the lymph nodes, but bone marrow, liver, and spleen involvement are frequently found at diagnosis.3,4 There was no or minimal lymphadenopathy, which was usually limited to hilar lymph nodes of the spleen. Histologically, micronodular THRBCL involves white pulp in a micronodular pattern. There are scattered large cells in a background of CD3+ T cells and CD68+ histiocytes. The large tumor cells express B-cell markers CD20 and bcl-6. They are variably positive for EMA, bcl-2, and CD30 and negative for CD10 and Epstein–Barr virus latent membrane proteins.1 THRBCL is considered an aggressive lymphoma, although clinical heterogeneity is described. The overall survival is about 50%, irrespective of histologic type. Recent studies suggest that splenectomy followed by combination chemotherapy may improve the survival. The combined morphologic, immunohistochemical, and molecular genetic data are essential for correct diagnosis of splenic T-cell-rich B-cell lymphoma of spleen.5
Please cite this article in press as: Panda S, et al. T-cell/histiocyte-rich B-cell lymphoma of spleen: A case report, Apollo Med. (2017), http:// dx.doi.org/10.1016/j.apme.2016.11.009
APME-393; No. of Pages 3 apollo medicine xxx (2017) xxx–xxx
3.
Conclusion
Indeed, splenic lymphomas (SLs) are composed of a wide and heterogeneous array of diseases, whose clinical behavior spans from indolent to highly aggressive. The clinical presentation of patients with SLs is varied, composing truly asymptomatic patients with isolated splenomegaly, patients in whom splenomegaly is associated with Peripheral Blood count changes, and patients reporting constitutional symptoms and/or abdominal discomfort secondary to massive spleen enlargement. Such nonspecific pictures often cause patients to undergo extensive investigation for infective, hepatic, or hemolytic diseases by physicians of diverse subspecialties before a lymphoid malignancy is suspected. Therefore, when approaching patients with suspected SLs, our primary effort is to achieve a diagnosis by integrating presenting clinical and laboratory data with imaging, PB count, and bone marrow assessment for correct diagnosis of splenic T-cell-rich B-cell lymphoma of spleen.5
references
1. Dogan A, Burke JS, Goteri G, Stitson RN, Wotherspoon AC, Jsaacson PG. Micronodular T-cell/histiocyte-rich large B-cell lymphoma of the spleen: histology, immunophenotype and differential diagnosis. Am J Surg Pathol. 2003;27(7): 903–911. 2. Turkoz HK, Polat N, Akin L, Ozcan D. Micronodular T-cell/ histiocyte-rich B-cell lymphoma of the spleen in a case of small lymphocytic lymphoma: a Richter's transformation. Ups J Med Sci. 2010;115(3):217–219. 3. De Wolf-Peters C, Delabie J, Campo E, et al. T-cell/histiocyterich large B-cell lymphoma. In: Swerdhow SH, Campo E, Harris NL, eds. et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue 4th ed. Lyon: IARC Press; 2008:238–239. 4. Dincol G, Agan M, Dogan O, Diz-Kucukkaya R, Uslu B. T-cell-rich B-cell lymphoma of the spleen presenting with severe hypersplenism. Clin Lab Haematol. 2006;28(6): 419–422. 5. Li S, Mann KP, Holden JT. T-cell-rich B-cell lymphoma presenting in the spleen: a clinicopathologic analysis of 3 cases. Int J Surg Pathol. 2004;12(1):31–37.
Conflicts of interest The authors have none to declare.
Please cite this article in press as: Panda S, et al. T-cell/histiocyte-rich B-cell lymphoma of spleen: A case report, Apollo Med. (2017), http:// dx.doi.org/10.1016/j.apme.2016.11.009
3
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/apme
Case Report
T-cell/histiocyte-rich B-cell lymphoma of spleen: A case report Srikanta Panda a, Roshni Tirkey b, Anshuman Sarangi c,* a
Associate Professor, P.G. Department of Surgery, S.C.B. Medical College and Hospital, Cuttack, Odisha, India Junior Resident, P.G. Department of Surgery, S.C.B. Medical College and Hospital, Cuttack, Odisha, India c Research Associate, Big Project, KIIT-TBI, KIIT University, Bhubaneswar, Odisha, India b
article info
abstract
Article history:
Splenomegaly is a frequent yet challenging condition that clinicians have to deal within
Received 26 October 2016
their daily clinical practice. Although the spleen is commonly involved in disseminated
Accepted 11 November 2016
lymphoma, primary lymphoma of the spleen is rare. A great majority of the reported cases
Available online xxx
are of B-cell lineage, with diffuse large B-cell lymphoma being the most common. A new variant of T-cell/histiocyte-rich B-cell was first described by Dogan et al. in 2003.1 The
Keywords:
combined morphologic, immunohistochemical, and molecular genetic data are essential for
Splenic lymphoma
a correct diagnosis of splenic T-cell-rich B-cell lymphoma. We report a classical case of T-
T-cell-rich B-cell lymphoma of
cell-rich B-cell lymphoma of the spleen in a 45-year-old male patient with clinical diagnosis
spleen
of splenomegaly. © 2017 Indraprastha Medical Corporation Ltd. All rights reserved.
Spleen Large B-cell lymphoma Splenomegaly
1.
Case report
A 45-year-old, Hindu male was presented with pain in the left upper abdomen that was spreading to the left shoulder since 6 months. On examination, a palpable left upper quadrant abdominal mass of 15 cm 18 cm was found in the supine and right lateral decubitus position with knees up and hips flexed. The mass extended from left the subcostal region up to the right iliac fossa slightly crossing the midline. Medial border and notch were palpable. On Ultrasound Sonography Test (USG) examination, a huge splenomegaly was confirmed (Figs. 1–4). Routine investigations were within normal limits. Vaccination against Pneumococcus, H-influenza type B, and
Meningococci type C were given 2 weeks prior to elective splenectomy. A left subcostal incision beginning two fingers breadth below the costal margin starting from the midline to the tip of the 11th costal cartilage was made. After operation, a huge spleen was isolated and sent for histopathologic examination to the Pathology Department, S.C.B. Medical College, Cuttack. Surgical pathology specimen received from splenectomy measured 23 cm 17 cm 11 cm with splenic notch. The specimen was grayish brown in color. Multiple sections were made from the cortex and the medulla. The histosections show splenic capsule with complete effacement of splenic architecture by small to intermediate to large, round to polygonal cells dispersed among the fibrocollagenous stroma
* Corresponding author. Tel.: +91 9861232063/9556078270. E-mail address: [email protected] (A. Sarangi). http://dx.doi.org/10.1016/j.apme.2016.11.009 0976-0016/© 2017 Indraprastha Medical Corporation Ltd. All rights reserved.
Please cite this article in press as: Panda S, et al. T-cell/histiocyte-rich B-cell lymphoma of spleen: A case report, Apollo Med. (2017), http:// dx.doi.org/10.1016/j.apme.2016.11.009
APME-393; No. of Pages 3
2
apollo medicine xxx (2017) xxx–xxx
Fig. 1 – Huge spleen after splenectomy.
Fig. 4 – Ki 67 positivity in less than 2% of the cells.
in vague follicular pattern. ImmunoHistoChemistry (IHC) for CDs shows that 80% of the cells are strongly positive. Cells are CD20 positive in large lymphocytes with vesicular nuclei constituting 10% of the cell population. Cells are also positive for bcl 6 and CD 79a but negative for CD 5, bcl-2, CD 10, CD 30, and CD 15. Ki-67 proliferation index was less than 2%, and in large cells it was 90%. With these findings, a diagnosis of T-
cell-rich B-cell lymphoma of spleen was given. Then the patient was advised an examination of the bone marrow, which did not reveal any infiltration by the lymphoma cells.
Fig. 2 – Haematoxylin and Eosin stain (HE) slide showing splenic lymphoma cells.
Fig. 3 – CD3 reactive proliferation of T-cells.
2.
Discussion
Incidental finding of an isolated splenomegaly during clinical assessment of patients evaluated for unrelated causes has become increasingly frequent because of the widespread use of imaging. A true diagnostic dilemma hides in distinguishing pathologic conditions primarily involving the spleen from those in which splenomegaly presents as an epiphenomenon of hepatic or systemic diseases.1,2 To qualify for primary splenic status, the lymphoma should be confined to the spleen and/or splenic hilar lymph nodes, although some authors also accept the presence of bone marrow involvement. Micronodular T-cell/histiocyte-rich B-cell (THRBCL) lymphoma of spleen was first reported and described by Dogan et al. in 2003.1 It is a type of Non-Hodgkin Lymphoma (NHL) characterized by a limited number of scattered large, atypical B-cells embedded in a background of abundant T-cells and frequently along with histiocytes. It accounts for less than 10% of all diffuse large B-cell lymphomas. It mainly affects the lymph nodes, but bone marrow, liver, and spleen involvement are frequently found at diagnosis.3,4 There was no or minimal lymphadenopathy, which was usually limited to hilar lymph nodes of the spleen. Histologically, micronodular THRBCL involves white pulp in a micronodular pattern. There are scattered large cells in a background of CD3+ T cells and CD68+ histiocytes. The large tumor cells express B-cell markers CD20 and bcl-6. They are variably positive for EMA, bcl-2, and CD30 and negative for CD10 and Epstein–Barr virus latent membrane proteins.1 THRBCL is considered an aggressive lymphoma, although clinical heterogeneity is described. The overall survival is about 50%, irrespective of histologic type. Recent studies suggest that splenectomy followed by combination chemotherapy may improve the survival. The combined morphologic, immunohistochemical, and molecular genetic data are essential for correct diagnosis of splenic T-cell-rich B-cell lymphoma of spleen.5
Please cite this article in press as: Panda S, et al. T-cell/histiocyte-rich B-cell lymphoma of spleen: A case report, Apollo Med. (2017), http:// dx.doi.org/10.1016/j.apme.2016.11.009
APME-393; No. of Pages 3 apollo medicine xxx (2017) xxx–xxx
3.
Conclusion
Indeed, splenic lymphomas (SLs) are composed of a wide and heterogeneous array of diseases, whose clinical behavior spans from indolent to highly aggressive. The clinical presentation of patients with SLs is varied, composing truly asymptomatic patients with isolated splenomegaly, patients in whom splenomegaly is associated with Peripheral Blood count changes, and patients reporting constitutional symptoms and/or abdominal discomfort secondary to massive spleen enlargement. Such nonspecific pictures often cause patients to undergo extensive investigation for infective, hepatic, or hemolytic diseases by physicians of diverse subspecialties before a lymphoid malignancy is suspected. Therefore, when approaching patients with suspected SLs, our primary effort is to achieve a diagnosis by integrating presenting clinical and laboratory data with imaging, PB count, and bone marrow assessment for correct diagnosis of splenic T-cell-rich B-cell lymphoma of spleen.5
references
1. Dogan A, Burke JS, Goteri G, Stitson RN, Wotherspoon AC, Jsaacson PG. Micronodular T-cell/histiocyte-rich large B-cell lymphoma of the spleen: histology, immunophenotype and differential diagnosis. Am J Surg Pathol. 2003;27(7): 903–911. 2. Turkoz HK, Polat N, Akin L, Ozcan D. Micronodular T-cell/ histiocyte-rich B-cell lymphoma of the spleen in a case of small lymphocytic lymphoma: a Richter's transformation. Ups J Med Sci. 2010;115(3):217–219. 3. De Wolf-Peters C, Delabie J, Campo E, et al. T-cell/histiocyterich large B-cell lymphoma. In: Swerdhow SH, Campo E, Harris NL, eds. et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue 4th ed. Lyon: IARC Press; 2008:238–239. 4. Dincol G, Agan M, Dogan O, Diz-Kucukkaya R, Uslu B. T-cell-rich B-cell lymphoma of the spleen presenting with severe hypersplenism. Clin Lab Haematol. 2006;28(6): 419–422. 5. Li S, Mann KP, Holden JT. T-cell-rich B-cell lymphoma presenting in the spleen: a clinicopathologic analysis of 3 cases. Int J Surg Pathol. 2004;12(1):31–37.
Conflicts of interest The authors have none to declare.
Please cite this article in press as: Panda S, et al. T-cell/histiocyte-rich B-cell lymphoma of spleen: A case report, Apollo Med. (2017), http:// dx.doi.org/10.1016/j.apme.2016.11.009
3