Rare Causes of Isolated and Progressive Splenic Lesions: Challenges in Differential Diagnosis, Evaluation, and Treatment of Primary Splenic Lymphomas (original) (raw)
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A Case Report on Splenic Marginal Zone Lymphoma
Journal of Pharmaceutical Research International, 2021
Background: Out of the various malignant tumours originating from the lymphatic hematopoietic system, lymphoma is one such important entity. It is divided into Non-Hodgkin’s Lymphoma (NHL) and Hodgkin Lymphoma (HL) depending on its cell source. A very rare type of malignant variant of lymphoma is the primary splenic lymphoma, involving exclusively the spleen and splenic hilar lymph nodes. Moreover, splenic marginal zone lymphoma (SMZL) is even more infrequent. SMZL is an uncommon chronic B lymphocyte proliferative disease, which only accounts for about 1–2% of all non-Hodgkin’s lymphoma. The mean age of SMZL incidence is about 65 years. There is no known significant gender predominance. A quarter of patients with early diagnosed SMZL have known to have vague symptoms like abdominal pain and distention; and other patients may be accompanied by loss of weight, malaise, cachexia, splenomegaly, or other manifestations. Conclusion: Although, a good prognostic outcome is what is usually e...
Non-Hodgkin’s Lymphoma of Spleen: A Rare Site of Primary Presentation
Journal of Case Reports, 2020
Background: Isolated splenic involvement in non-Hodgkin's lymphoma is a rare clinical entity and is termed as primary splenic lymphoma (PSL). In such cases, the disease is mostly confined to spleen and splenic hilar lymph nodes. It usually presents with dragging pain in left side of abdomen and huge splenomegaly is detected on investigations. Splenectomy is the main modality of treatment followed by chemotherapy with or without radiation. Case Report: We present a rare case of PSL treated successfully at our institution with splenectomy and chemotherapy. Conclusion: Primary splenic lymphoma is a rare splenic neoplasm. Immunohistochemistry markers are helpful in confirming the diagnosis. Splenectomy is both diagnostic and therapeutic in such cases.
Primary splenic diffuse large B-cell lymphoma: a case report
European Journal of Case Reports in Internal Medicine, 2023
Primary splenic lymphoma (PSL) is a rare disease and an improbable cause of splenomegaly or splenic nodules. On the contrary, splenic secondary involvement as part of an advanced lymphoproliferative disorder is more common. The authors present the case of a 49-year-old woman with a primary splenic diffuse large B-cell lymphoma (PS-DLBCL), in which the absence of other organs' involvement determined an ultrasound-guided biopsy of the spleen to achieve a definitive diagnosis. With this case the authors intend to emphasise the extensive differential diagnosis of splenomegaly, splenic nodules or infiltrates, the usefulness of splenic biopsy in establishing the diagnosis and recall a rare disease, with non-specific presenting symptoms, in which the diagnostic workup is challenging.
Primary non-Hodgkin lymphoma of the spleen
American Journal of Hematology, 1991
Primary splenic lymphoma is a relatively infrequent cause of splenomegaly, its reported incidence being less than 1%. Nonetheless, various series have reported on its occurrence and its unfavorable prognosis, but these series have reported data on heterogenous populations, including many patients with nodal or hepatic disease. We report on a series of nine patients with primary splenic lymphoma. None of these patients had biopsy-proven evidence of extrasplenic disease and were categorized as involving spleen only (stage 1) or spleen and splenic hilum (stage 2). In this series, classified by the recent N.C.I. working formulation, four patients had intermediate or high-grade histology and five patients had low-grade histology. There was no correlation between histological subtype and prognosis, nor could other factors be delineated to explain their favorable prognosis. The median survival in this group of patients was 7.48 years, and no evidence of relapse has been documented. The data suggest that primary splenic lymphoma, treated by splenectomy alone or combination therapy, may be potentially curative. Further studies should address the question of aggressive accurate staging in hopes of obtaining homogenous patient populations so that appropriate treatment in primary splenic lymphoma can be better defined .
https://www.ijhsr.org/IJHSR\_Vol.7\_Issue.3\_March2017/IJHSR\_Abstract.053.html, 2017
Splenic lymphoma is an indolent form of mature B-cell neoplasm. It is very rare and overlaps with other lymphomas. The hall mark of clinical presentation is splenomegaly and it usually becomes symptomatic when spleen becomes massive and associated with cytopenia. We discuss here about a 48yr old female with malignant non Hodgkin's lymphoma-spleen. The case is published for its rarity.
IOSR Journal of Dental and Medical Sciences, 2016
Splenic marginal zone lymphoma(SMZL) is a rare B-cell neoplasm comprising less than 2% of lymphoid neoplasms ,composed of small lymphocytes which surround and replace the splenic white pulp ,germinal centres,follicle mantle and merge with marginal zone.We report a case of SMZL in a 40 year old male patient presenting with complaints of low grade fever and left sided abdominal discomfort for 6 months and was noted to have massive splenomegaly on physical examination. Laboratory evaluation revealed leucocytosis and presence of atypical lymphocytes which were larger than mature lymphocytes having moderately basophilic cytoplasm with cytoplasmic processes at the poles of the cell with round to oval nucleus with clumped chromatin and indistinct nucleoli.Immunophenotypic analysis revealed a B-cell population with CD-20 positivity.
Unusual Course of Splenic Marginal Zone Lymphoma
World Journal of Oncology, 2013
A 53-year-old woman was diagnosed with splenic marginal zone lymphoma by pathological examination on left submandibular lymph node and bone marrow biopsies and markedly enlarged spleen. Four cycles of Rituximab chemotherapy were given. Seven months after finishing Rituximab chemotherapy, she developed left upper extremity swelling without evidence of deep venous thrombosis. Repeat PET/CT scan demonstrated multiple left axillary lymph nodes extending to left retroclavicular region and a new lymph node posterior to the left scapula. Biopsy of the lymph node demonstrated marginal zone lymhoma pattern with increased numbers of large cells, but not outright diffuse large B-cell lymphoma. Despite resuming rituximab, patient had persistent leukocytosis and severe anemia. Restaging PET/CT showed 3 new left anterior cervical lymph nodes and 1 new right axillary lymph node. Spleen has further enlarged. R-CHOP chemotherapy was started, which improved leukocytosis. After 4 cycles of R-CHOP, PET/CT showed new metabolic activity within right inguinal and abdominal lymph nodes. Patient was given one cycle of Bendamustine. She developed a possible "hematoma" in right medial elbow. However, MRI study revealed a subcutaneous deposit of the lymphoma. Patient needs consistently blood transfusion and she deteriorated quickly. Our patient had an aggressive course of splenic marginal zone lymphoma, not responding to four trials of chemotherapy although SMZL is well-known to be an indolent low grade lymphoma. This case report emphasizes the importance to individualize the treatment in SMZL patients and repeat bone marrow biopsy if the disease recurs.
How I diagnose and treat splenic lymphomas
Blood, 2011
The 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. Therefore, the challenging approach to the differential diagnosis of spleen disorders has emerged as a rather common issue of clinical practice. 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. Among the causes of isolated splenomegaly, lymphoid malignancies account for a relevant, yet probably underestimated, number of cases. Splenic lymphomas constitute a wide and heterogeneous array of diseases, whose clinical behavior spans from indolent to highly aggressive. Such a clinical heterogeneity is paralleled by the high degree of biologic variation in the lymphoid populations from which they originate. Nevertheless, the presenting clinical, labor...
Surgical approach of primary splenic lymphoma: report of a case and review of the literature
Indian Journal of Hematology and Blood Transfusion, 2009
A 80-year-old woman was hospitalized due to anemia and fever. Computed tomography depicted a solitary concentric, sharply marginated lesion of the spleen. Laparotomy identifi ed a creamy-white soft tumor. Histopathological examination confi rmed the diagnosis of non-Hodgkin B-cells anaplastic lymphoma. Extended range of pathological manifestations explains the variety of radiological appearances and diffi culty in accurate diagnostic process of primary splenic lymphoma.
PRIMARY LYMPHOMA OF THE SPLEEN. REPORT OF A CASE WITH DIAGNOSIS BY FINE-NEEDLE GUIDED BIOPSY
1995
Primary splenic lymphoma is a relatively infrequent disease; the diagnosis of this entity is cur- rently made with splenectomy. In a 52-year-old female with left upper quadrant abdominal pain, ultrasound showed a normal-sized spleen with an internal hypoechoic focal lesion. Ultrasonically- guided fine-needle aspiration and tissue core biopsy of the splenic lesion showed non-Hodgkin's lymphoma (NHL). At the time of presentation there was no evidence of involvement of lymph nodes, bone marrow or any other organ. A diagnosis of primary splenic non-Hodgkin's lymphoma was made and the patient underwent laparotomy with splenectomy. Histologic examination of the spleen confirmed the diagnosis: low-grade NHL confined to the spleen. The patient is well and in complete remission seven months after diagnosis. The purpose of this paper is to report a rare occurrence of primary splenic lymphoma and to demonstrate the possibility of making this diagnosis by percutaneous guided biopsy.