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CASE REPORT
Year : 2016  |  Volume : 5  |  Issue : 1  |  Page : 59-62

A rare case of primary follicular lymphoma of the spleen


1 Department of Medicine, Division of Oncology, GSL Medical College and General Hospital, Rajahmundry, Andhra Pradesh, India
2 Department of Surgery, Division of Oncology, GSL Medical College and General Hospital, Rajahmundry, Andhra Pradesh, India

Date of Web Publication18-Mar-2016

Correspondence Address:
Manne Sriharibabu
Department of Medicine, GSL Medical College and General Hospital, NH-16, Rajahmundry - 553 296, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.178982

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  Abstract 

Primary splenic lymphoma (PSL) is a rare entity accounting for less than 1% of lymphoid malignancies. Follicular lymphoma primarily involving the spleen is still rare and constitutes only 10% of the PSLs. PSLs constitute a heterogeneous group of diseases with wide variations in clinical presentation as well as lymphoid populations from which they originate. Here we are reporting a case of a 43-year-old female presenting with massive splenomegaly and hypersplenism with anemia, leucopenia, and thrombocytopenia. Other investigations revealed no abnormality. When the patient underwent splenectomy for hypersplenism and the specimen was subjected to histopathological examination and immunohistochemistry the diagnosis of follicular lymphoma was made. As splenomegaly and hypersplenism are common in clinical practice, other common causes of splenomegaly and hypersplenism should be excluded. High level of suspicion and thorough investigation are required to establish the diagnosis. In the present case, the diagnosis was made retrospectively after splenectomy.

Keywords: Follicular lymphoma, involved field radiotherapy (IFRT), immunochemotherapy, immunohistochemistry, primary splenic lymphoma (PSL)


How to cite this article:
Sarma YS, Sriharibabu M, Nayak SR, Spoorthy K. A rare case of primary follicular lymphoma of the spleen . J NTR Univ Health Sci 2016;5:59-62

How to cite this URL:
Sarma YS, Sriharibabu M, Nayak SR, Spoorthy K. A rare case of primary follicular lymphoma of the spleen . J NTR Univ Health Sci [serial online] 2016 [cited 2020 Mar 31];5:59-62. Available from: http://www.jdrntruhs.org/text.asp?2016/5/1/59/178982


  Introduction Top


Primary splenic lymphoma (PSL) is a rare entity accounting for less than 1% of lymphoid malignancies. Even though splenic involvement is common in lymphoid malignancies, isolated splenic involvement as the presenting feature of lymphoma is rare. [1],[2],[3] When lymphoma is confined to spleen and its hilum, it is called PSL. [4] PSLs constitute a heterogeneous group of diseases with wide variations in clinical presentation as well as lymphoid populations from which they originate. The PSLs include splenic marginal zone lymphoma, hairy cell leukemia, hairy cell variant, splenic diffuse red pulp B-cell lymphoma, lymphoplasmacytic lymphoma, primary splenic follicular lymphoma, B- and T-prolymphocytic leukemia, large granular lymphocytic leukemia, and hepatosplenic T-cell lymphoma. The patients with PSL may be asymptomatic and present with abdominal discomfort secondary to splenomegaly, constitutional symptoms, or with changes in peripheral blood picture. The diagnostic work-up includes exclusion of infective, hepatic, hemolytic, and autoimmune causes of splenomegaly followed by peripheral blood and bone marrow examination. Ultrasound imaging of the abdomen and whole body computed tomography scan are necessary to assess the involvement of other organs. [5] Here we are reporting a case of a 43-year-old lady who presented with unexplained splenomegaly and finally diagnosed as primary follicular lymphoma of the spleen.


  Case report Top


A middle-aged female patient was admitted to our hospital with 3 years history of slow growing abdominal mass involving left hypochondrium and umbilical region. Initially, the swelling was painless but as it enlarged in size the patient developed pain and discomfort and sought medical advice. Clinical examination revealed massive splenomegaly with no hepatomegaly or lymphadenopathy. There was no history of fever, loss of appetite, or weight loss and the patient was found to be moderately anemic. Her pulse rate was 92/min and her blood pressure was 110/70 mmHg. Her hemoglobin was 8.4 gm%/dL, total leukocyte count was 2,700/cumm, and her platelet count was 1.1 lakhs. Serology for hepatitis B, C, and human immunodeficiency virus (HIV) was negative. Coomb's test and tests for serum M component, rheumatoid factor, and anti nuclear antibody (ANA) were negative. Peripheral blood smear examination revealed mild dimorphic picture with evidence of hemolysis and leucopenia with no abnormal cells or parasites. Hepatic and renal parameters were normal and bone marrow examination revealed erythroid hyperplasia. Ultrasound imaging of the abdomen and whole body computed tomography scan revealed splenomegaly with no other abnormality. Initial diagnosis was splenomegaly with hypersplenism. The patient was advised to undergo splenectomy. At splenectomy, the size of the spleen was 20 × 15 × 10 cm, and the surface was studded with multiple small gray white nodules [Figure 1]a and b]. Microscopic examination of the splenic specimen revealed expanded white pulp with large round-to-irregular discrete coalescent nodules made up of small-to-medium-sized neoplastic lymphoid cells [Figure 2]. These cells showed round-to-angulated nuclei and scanty-to-moderate eosinophilic cytoplasm with vacuoles in the cytoplasm of some of these cells [Figure 3]. The compressed red pulp was seen as intersecting bundles separating the nodules. The sinusoids in the red pulp were dilated and congested with lymphoid cell infiltration [Figure 4]. The histopathological diagnosis was a non-Hodgkin's lymphoma of the spleen-Follicular type. On immunohistochemical analysis, the tumor cells were CD10+ and BCL2+ phenotype and the final diagnosis was non-Hodgkin's lymphoma of the spleen follicular type grade I.
Figure 1: (a) Gross view of the splenic specimen with multiple gray white nodules on its surface. (b) Cut section of the splenic specimen studded with gray white nodules

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Figure 2: Expanded white pulp with compressed red pulp looking like intersecting bundles

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Figure 3: Microscopic view of a nodule made up of small-tomedium sized lymphoid cells with round-to-angulated nuclei and moderate-to-scanty eosinophilic cytoplasm with vacuoles (H and E stain, ×100)

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Figure 4: Red pulp shows dilated and congested sinusoids infiltrated with lymphoid cells

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  Discussion Top


Splenic marginal zone lymphoma and splenic diffuse red pulp B-cell lymphoma account for nearly 80% of isolated splenic lymphomas. Primary follicular lymphoma of the spleen is uncommon and in a study involving 49 cases PSLs, follicular lymphoma was seen in only 10% of cases. [6] Follicular lymphomas are malignant counterparts of germinal center B cells. The cytogenetic hallmark of follicular lymphoma is translocation between chromosome 14 and 18 that is seen in 80-90% of cases. The molecular consequence of this translocation is the juxtaposition of B cell lymphoma/leukemia 2 proto-oncogene with enhancer sequences of the immunoglobulin heavy chain gene promoter region resulting in overexpression of BCL2 protein. Overexpression of BCL2 protein of the mitochondrial membrane prevents cells in follicular center from undergoing apoptosis. [7]

Follicular lymphoma involving the spleen may show two distinct histological patterns with either preservation or disruption of the splenic architecture. In the former, only scattered neoplastic germinal centers with an entirely intrafollicular growth pattern and retention of the overall splenic architecture is seen. In the later aggregates of closely packed neoplastic follicles, often enlarged and disrupting the splenic architecture and frequently show an interfollicular proliferation of neoplastic B cells. Splenic follicular lymphoma may exhibit same morphologic, phenotypic, and cytogenetic findings similar to that of nodal follicular lymphoma but purely intrafollicular growth pattern may easily be misdiagnosed as reactive hyperplasia. Careful morphologic evaluation of atypical-appearing germinal centers and appropriate use of immunohistochemical stains, especially for CD10+ and BCL2, will assist in confirming the diagnosis. [8]

This case of small cell follicular lymphoma with preserved splenic architecture was diagnosed by the presence of nodular growths in white pulp composed of a relatively pure population of small-to-medium sized lymphocytes and infiltration of the red pulp by the same cells that helped in distinguishing lymphoma from benign reactive hyperplasia. Immunohistochemistry helped in confirming the diagnosis and the final diagnosis of non-Hodgkin's lymphoma of the spleen, follicular type grade 1, and clinical stage 1 was made as large transformed cells or centroblasts were not seen and as the disease was confined to spleen only.

Follicular lymphoma is graded basing on the absolute number of centroblasts seen across 10 representative neoplastic follicles. It is grade 1 when the number of centroblasts is 0-5/high-power field, grade 2 when the number of centroblasts is 6-15 and grade 3a when the number of centroblasts exceeds 15, but centrocytes are seen and grade 3b when solid sheets of centroblasts are seen. The prognosis of PSL depends on the histological type, proliferative index, and its clinical stage. [6],[9] When the PSL is confined to spleen it is stage 1, when it extends to hilar lymph nodes it is stage 2, and when it involves other abdominal lymph glands and viscera it is stage 3.

The treatment options for follicular lymphoma vary from individual to individual depending upon the age of the patient, extent of the disease, presence of the comorbid conditions, and goals of the therapy. [10] The treatment options for stages 1 and 2 of the disease include involved field radiotherapy (IFRT), immunotherapy, chemotherapy, chemoimmunotherapy, and radio immune therapy (RIT). However, while choosing initial therapy care should be taken to avoid excessively myelotoxic regimens as subsequently these patients may need high dose therapy followed by autologous stem cell rescue (HDT/ASCR). As the present case was diagnosed after splenectomy, the patient was treated with Cyclophosphamide, Adriamycin, Vincristine, and Prednisolone (CHOP) regimen as per the provisions of governmental Health Insurance Scheme (Rajiv Arogyasri) under which the patient was enrolled for the treatment.


  Conclusion Top


In conclusion, primary lymphoma of the spleen, particularly follicular lymphoma, is a very uncommon entity even though spleen is commonly involved in lymphomas. The clinical presentation is characterized by nonspecific symptoms and signs. The diagnosis is made only after the exclusion of other common causes of splenomegaly and hypersplenism and requires thorough investigation. In most of the cases reported in medical literature, such as the present case, the diagnosis was made retrospectively after splenectomy.

Financial support and sponsorship

The authors disclose that this article received no funding from any source.

Conflicts of interest

The authors declare no potential conflict of interest with respect to the authorship and/or publication of this article.

 
  References Top

1.
Koiso H, Yokohama A, Mitsui T, Saitoh T, Handa H, Murakami H, et al. Follicular lymphoma presenting with marked splenomegaly: Report of three cases. Acta Haematol 2012;128:47-52.  Back to cited text no. 1
    
2.
Nair S, Shukla J, Chandy M. Non-Hodgkin's lymphoma presenting with prominent splenomegaly; clinicopathologic diversity in relationship to immunophenotype. Acta Oncol 1997;36:725-7.  Back to cited text no. 2
    
3.
Abraksia S, Kumar PD, Kasal J. Two unusual lymphomas. Case 1: Primary malignant lymphoma (diffuse large B-cell lymphoma) of the spleen mimicking splenic abscess. J Clin Oncol 2000;18:3731-3.  Back to cited text no. 3
    
4.
Gobbi PG, Grignani GE, Pozzetti U, Bertoloni D, Pieresca C, Montagna G, et al. Primary splenic lymphoma: Does it exist? Haematologica 1994;79:286-93.  Back to cited text no. 4
    
5.
Iannitto E, Tripodo C. How I diagnose and treat splenic lymphomas. Blood 2011;117:2585-95.  Back to cited text no. 5
    
6.
Kraemer BB, Osborne BM, Butler JJ. Primary splenic presentation of malignant lymphoma and related disorders. A study of 49 cases. Cancer 1984;54:1606-19.  Back to cited text no. 6
[PUBMED]    
7.
Ott G, Rosenwald A. Molecular pathogenesis of follicular lymphoma. Haematologica 2008;93:1773-6.  Back to cited text no. 7
[PUBMED]    
8.
Howard MT, Dufresne S, Swerdlow SH, Cook JR. Follicular lymphoma of the spleen: Multiparameter analysis of 16 cases. Am J Clin Pathol 2009;131:656-62.  Back to cited text no. 8
    
9.
Mollejo M, Rodríguez-Pinilla MS, Montes-Moreno S, Algara P, Dogan A, Cigudosa JC, et al. Splenic follicular lymphoma: Clinicopathologic characteristics of a series of 32 cases. Am J Surg Pathol 2009;33:730-8.   Back to cited text no. 9
    
10.
Musteata VG, Corcimaru IT, Iacovleva IA, Musteata LZ, Suharschii IS, Antoci LT. Treatment options for primary splenic low-grade non-Hodgkin's lymphomas. Clin Lab Haematol 2004;26:397-401.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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