Smzl lymphomagenesis involves antigen andor superantigen stimulation and molecular deregulation of genes notch2 and klf2 involved in the physiological differentiation of spleen marginal zone b cells. Short surface cellular expansions were observed on blood and marrow films and by transmission electron microscopy. The association between hepatitis c and bcell nonhodgkin lymphoma nhl has been suggested by several studies. Splenic lymphoma with villous lymphocytes slvl is a lowgrade disorder that regularly presents. Splenic marginal zone bcell lymphoma demonstrating the expansion of marginal zone of the white pulp in the spleen. Splenic marginal zone lymphoma with villous lymphocytes shows. A 49yearold woman presented with splenic lymphoma with villous lymphocytes slvl that showed a clonal abnormality of del7q22q32 in addition to inv10pq23, the latter being a previously. The immunophenotype of splenic lymphoma with villous. Primary splenic lymphoma is a rare entity, has vague clinical presentation and can present with grave complications like hypersplenism and splenic rupture. Emergency splenectomy is an effective therapeutic and diagnostic tool in cases with massive splenomegaly with. The immunophenotype was that of mature b cells without cd5, cd10, cdllc, or cd25 expression or tartrateresistant acid phosphatase. Extranodal sites and lymph nodes may also be involved. A singlecenter experience in splenic diffuse red pulp.
Splenic marginal zone lymphoma with and without villous. Cytogenetic studies in splenic lymphoma with villous lymphocytes. Splenic marginal zone lymphoma with villous lymphocytes. The neoplastic cells are both small lymphocytes and larger, transformed lymphoblasts, and they invade the mantle zone of splenic follicles and erode the marginal zone. Cell membrane markers are those of a b mature cell, and no particular chromosomal abnormalities have been associated with this disease. Splenic lymphoma with villous lymphocytes is a new entity characterised by the presence of atypical lymphocytes in the peripheral blood and bone marrow, and splenic infiltration in the white and red pulp. Splenic marginal zone lymphoma with villous lymphocytes shows ongoing immunoglobulin gene mutations. Pathology outlines splenic lymphoma with villous lymphocytes. Systemic mastocytosis associated with splenic marginal zone.
Diagnosis requires either spleen histology or, alternatively, the. Traverseglehen a, baseggio l, bauchu ec, morel d, gazzo s, ffrench m, et al. Splenic marginal zone lymphoma smzl or splenic lymphoma with villous lymphocytes is a bcell neoplasm of small to mediumsized lymphocytes presumably. Pdf outline for writing an article for current treatment. Splenic marginal zone lymphoma smzl is a rare form of indolent b. Rituximab, used alone or in combination, is superior to. Older names include lymphoma simulating hairy cell leukemia and lymphoplasmacytic lymphoma with circulating villous lymphocytes. Splenic lymphoma with villous lymphocytes gupta r, naseem s. Mature nuclear features are present, and many cells have abundant cytoplasm. Clinical features of splenic lymphoma with villous lymphocytes slvl th e re al incid enc e of slvl ha s never been calcul at ed, even if it has been esti mate d as les s tha n 2% of all non.
Splenic b cell lymphoma with circulating villous lymphocytes. Whether this condition is identical to splenic marginal zone lymphoma, or only highly similar, is a matter of debate. In west africa, splenic lymphoma with villous lymphocytes and hyperreactive malarial splenomegaly have many clinical and immunological features in common suggesting an aetiopathological link. Regression of splenic lymphoma with villous lymphocytes after treatment of hepatitis c virus infection hermine o, lefrere f, bronowicki jp, mariette x, jondeau k, eclachesaudreau v, et al. Polymorphous population with mantle and marginal zone distribution. Rare disorder, usually considered the leukemic variant of splenic marginal zone b cell lymphoma. Treatment of splenic marginal zone lymphoma with rituximab. Splenic lymphoma with villous lymphocytes slvl in 2005. Splenic lymphoma with villous lymphocytes slvl is a recently identified b cell lymphoproliferative disorder characterised by splenomegaly and the presence in the peripheral blood of lymphocytes. Nine patients who had splenic lymphoma with villous lymphocytes and hcv infection were treated with interferon alfa2b 3 million iu three times per week alone or in combination with ribavirin.
The presence of circulating villous lymphocytes vls in lymphoma patients usually points to splenic marginal zone bcell lymphoma smzl, even if the vls can be found occasionally in other small bcell lymphomas. The diagnosis of splenic lymphoma with villous lymphocytes was based on the presence of typical clinical, hematologic, and immunologic findings as previously described. Rare disorder, usually considered the leukemic variant of splenic marginal zone b cell lymphoma clinical features. Splenic lymphoma with circulating villous lymphocytes splenic marginalzone lymphoma. Close to a third of the patients do not require intervention and a policy of watch and see is reasonable and. Splenic lymphoma with villous lymphocytes slvl is a low. Splenic lymphoma with villous lymphocytes slvl is a recently recognized entity among chronic b lymphoproliferative disorders.
Splenic lymphoma with villous lymphocytes an overview. Splenic lymphoma with villous lymphocytes semantic scholar. Pdf splenic lymphoma with villous lymphocytes slvl is a lowgrade disorder that regularly presents with peripheral blood involvement. Splenic marginal zone b cell lymphoma stanford university. Splenic lymphoma, villous lymphocytes, leukemia abstract splenic lymphoma with villous lymphocytes slvl is a rare lowgrade b cell nonhodgkins lymphoma with distinct clinical and morphologic features.
What is splenic marginal zone lymphoma lymphoma is a type of blood cancer that develops when white blood cells called lymphocytes grow out of control. Splenic lymphoma with villous lymphocytes presenting as. How i diagnose and treat splenic lymphomas blood american. The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other bcell disorders by estella matutes, ricardo morilla, kwasi owusuankomah, angela houlihan, and daniel catovsky splenic lymphoma with villous lymphocytes slvl is a. The histopathology of splenic lymphoma with villous lymphocytes. Splenic marginal zone lymphoma with villous lymphocytes peripheral blood 3. Differential diagnosis of b cell leukaemias with large spleens article pdf available in journal of clinical pathology 406. Diagnosis of splenic bcell lymphomas in the bone marrow. Lymphomawith villous lymphocytes platelets white cells lymphoidcells villous lymphocytes x 109l %white cells paraprotein class gil 116 11 92 82 nottested 205 16 11 0 50 nottested 71 15 147 84 igmserum 16 5 notrecorded 68 34 igmserum 48 99 10 1 72 none 148 11 58 28 kufine 120 20 18 6 80 igmserum 45 kurine. Splenic diffuse red pulp lymphoma, splenic lymphoma, splenic marginal cell lymphoma introduction the spleen can be involved in the pathologic process of a variety. The histopathology of splenic lymphoma with villous. It is the leukemic counterpart of splenic marginal zone lymphoma smzl and is characterized by splenomegaly, often with no lymphadenopathy, moderate.
Regression of splenic lymphoma with villous lymphocytes after. Villous lymphocytes in splenic large bcell lymphoma with. Peripheral blood lymphocytosis in 75% of patients and villous lymphocytes on. Mar 03, 2011 highgrade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes. In patients with psl, therapeutic splenectomy is to be done. Characteristic features include splenomegaly, bone marrow infiltration with intrasinusoidal. Lymphomawith villous lymphocytes phasewas raised by the referring physician. Pdf splenic red pulp lymphoma with numerous basophilic. Splenectomy andor chlorambucil are usually regarded as the most effective treatment in slvl. Splenic marginal zone lymphoma smzl is a type of cancer specifically a lymphoma made up of bcells that replace the normal architecture of the white pulp of the spleen. The histopathology of splenic lymphoma with villous lymphocytes pg isaacson.
We report a case in a 55 year old man with the diagnosis of chronic lymphocytic leukemia. Pdf the histopathology of splenic lymphoma with villous. This report describes the occurrence of splenic lymphoma with villous lymphocytes slvl in a 56 year old white female with a family history of chronic lymphocytic leukaemia. Regression of splenic lymphoma with villous lymphocytes. Bhs guidelines for the treatment of marginal zone lymphomas. We identified lymphoma cases with numerous basophilic vls among the large group. Splenic mzl might not cause any symptoms at all but your doctor might notice that your spleen is bigger than it should be, or find abnormal cells when you have a blood test done for another reason. However, those cells are variably described, and detailed cytologic characterization is often lacking. Primary splenic lymphoma psl is very unusual entities if strict diagnostic criteria are applied. Splenic lymphoma with villous lymphocytes request pdf. Splenic lymphoma with villous lymphocytes slvl responding to 2chlorodeoxyadenosine 2cda. Small round lymphocytes surround and replace follicles follicular colonization results in micronodular pattern. They travel around your body in your lymphatic system, helping you fight infections. Other unusual features included a marked lymphocytosis with counts up to 224 x 109l and marked clumping of lymphocytes in edta anticoagulated blood.
Splenic lymphoma with circulating villous lymphocytes. A singlecenter experience in splenic diffuse red pulp lymphoma diagnosis. Splenic marginal zone lymphoma an overview sciencedirect. Prognostic features of splenic lymphoma with villous. Splenic marginal zone lymphoma smzl is a rare indolent lymphoma subtype which accounts for monoclonal gammopathy in extranodal marginal zone. The different diagnosis, work up and treatment options are discussed in these guidelines. The histopathology of splenic lymphoma with villous lymphocytes article pdf available in blood 8411. Tumours of this type are generally localised to the spleenwhich is commonly the only visible site of diseasebut can involve the bone marrow and blood. Splenic lymphoma with villous lymphocytes slvl in 2005, authors. Splenic lymphoma with villous lymphocytes wikipedia.
Splenic lymphoma with villous lymphocytes slvl is a recently identified b cell lymphoproliferative disorder characterised by splenomegaly and the presence in the peripheral blood of lymphocytes w. Splenic lymphoma with villous lymphocytes slvl is a rare lowgrade b cell nonhodgkins lymphoma with distinct clinical and morphologic features. Monoclonal gammopathies in bcell nonhodgkins lymphomas. Splenic lymphoma with villous lymphocytes slvl author. Cytomorphologic findings can be similar to those of splenic marginal zone lymphoma. Splenic b cell lymphoma with lymphocyte clusters in. In the world health organization classification system, splenic marginalzone lymphoma splenic mzl is described as an indolent bcell lymphoma, which generally presents as splenomegaly with involvement of the bone marrow and peripheral blood. Highgrade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes. Splenic lymphoma with villous lymphocytes pathology outlines. Pdf splenic b cell lymphoma with circulating villous.
Presenting features and response to therapy have been described, but information on prognostic factors is scanty. In such circumstances, core biopsyfine needle aspiration cytology can hit the correct pathological diagnosis. The immunophenotype was consistent with a diagnosis of splenic lymphoma with villous lymphocytes slvl. Splenic lymphoma with villous lymphocytes is a rare type of lymphoma that involves mature b cells. Pdf the immunophenotype of splenic lymphoma with villous. Renal biopsy demonstrated a membranoproliferative type i glomerulonephritis with intraluminal igg and igm deposits, and a skin biopsy showed a leucocytoclastic vasculitis with. Characteristic features include splenomegaly, bone marrow infiltration with. Case report splenic lymphoma with villous lymphocytes. Splenic lymphoma with villous lymphocytes in two sisters. Splenic mzl is characterised by micronodular infiltration of the. Splenic marginal zone lymphoma smzl or splenic lymphoma with villous lymphocytes is a bcell neoplasm of small to mediumsized lymphocytes presumably arising from the marginal zone of the splenic white pulp.
Review on splenic lymphoma with villous lymphocytes slvl, with data on clinics, and the genes involved. Tissue immunophenotyping was performed in 24 cases, 6 of which had frozen tissue. Presence of disease in peripheral lymph nodes and extranodal locations is uncommon. American society of hematology 2021 l street nw, suite 900, washington, dc 20036. As per previous studies the final diagnosis of psl should be made only when the disease is limited to spleen or involving hilar lymph nodes without any recurrence after splenectomy14. Oscier dg, matutes e, gardiner a, glide s, mould s, britobabapulle v, ellis j, catovsky d. Examination of the peripheral blood of patients with splenic lymphoma is useful because many patients with splenic marginal zone lymphoma have the peripheral blood changes of the clinical syndrome termed splenic lymphoma with villous lymphocytes slvl fig. Splenic red pulp lymphoma with numerous basophilic villous lymphocytes. Splenic lymphoma with villous lymphocytes slvl is a b cell chronic lymphoproliferative disorder. Clumping of lymphocytes in the presence of edta has been reported in a single case of chronic lymphocytic leukaemia cll, two cases of splenic lymphoma with villous lymphocytes slvl, and in benign lymphocytes. Prognostic features of splenic lymphoma with villous lymphocytes. It has a distinct clinical, morphological and immunophenotypic pattern and was previously described under a variety of designations. Splenic marginal zone lymphoma smzl is a rare bcell malignancy involving the spleen, bone marrow, and frequently the blood.
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