Case report splenic lymphoma with villous lymphocytes. In west africa, splenic lymphoma with villous lymphocytes and hyperreactive malarial splenomegaly have many clinical and immunological features in common suggesting an aetiopathological link. Splenic diffuse red pulp lymphoma, splenic lymphoma, splenic marginal cell lymphoma introduction the spleen can be involved in the pathologic process of a variety. Splenic marginal zone lymphoma smzl is a rare bcell malignancy involving the spleen, bone marrow, and frequently the blood. Splenic marginal zone bcell lymphoma demonstrating the expansion of marginal zone of the white pulp in the spleen. Splenic lymphoma with villous lymphocytes slvl in 2005, authors. Rare disorder, usually considered the leukemic variant of splenic marginal zone b cell lymphoma. Prognostic features of splenic lymphoma with villous. Pdf splenic lymphoma with villous lymphocytes slvl is a lowgrade disorder that regularly presents with peripheral blood involvement. 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.
Primary splenic lymphoma is a rare entity, has vague clinical presentation and can present with grave complications like hypersplenism and splenic rupture. Splenic marginal zone lymphoma with villous lymphocytes. Splenic lymphoma with villous lymphocytes request pdf. Emergency splenectomy is an effective therapeutic and diagnostic tool in cases with massive splenomegaly with. The association between hepatitis c and bcell nonhodgkin lymphoma nhl has been suggested by several studies. It has a distinct clinical, morphological and immunophenotypic pattern and was previously described under a variety of designations. 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 is a rare type of lymphoma that involves mature b cells. Splenic lymphoma with villous lymphocytes slvl is a b cell chronic lymphoproliferative disorder. In patients with psl, therapeutic splenectomy is to be done. In such circumstances, core biopsyfine needle aspiration cytology can hit the correct pathological diagnosis.
The histopathology of splenic lymphoma with villous. Splenic marginal zone lymphoma smzl or splenic lymphoma with villous lymphocytes is a bcell neoplasm of small to mediumsized lymphocytes presumably. Splenic marginal zone lymphoma smzl, a lymphoma previously also described as splenic lymphoma with villous lymphocytes, is usually characterized by variable numbers of circulating lymphoma cells with fine cytoplasmic projections, intrasinusoidal bone marrow involvement, and monoclonal gammopathy in onethird of the patients. Splenic mzl is defined as a bcell neoplasm consisting of a proliferation of small lymphocytes that surround and replace the splenic white pulp tissue. They travel around your body in your lymphatic system, helping you fight infections. Splenic red pulp lymphoma with numerous basophilic villous lymphocytes. 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.
Other unusual features included a marked lymphocytosis with counts up to 224 x 109l and marked clumping of lymphocytes in edta anticoagulated blood. Prognostic features of splenic lymphoma with villous lymphocytes. Splenic lymphoma with villous lymphocytes wikipedia. 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 lymphoma with villous lymphocytes in two sisters. Mature nuclear features are present, and many cells have abundant cytoplasm. Peripheral blood lymphocytosis in 75% of patients and villous lymphocytes on. Characteristic features include splenomegaly, bone marrow infiltration with. A singlecenter experience in splenic diffuse red pulp. Characteristic features include splenomegaly, bone marrow infiltration with intrasinusoidal. American society of hematology 2021 l street nw, suite 900, washington, dc 20036. Short surface cellular expansions were observed on blood and marrow films and by transmission electron microscopy. Primary splenic lymphoma psl is very unusual entities if strict diagnostic criteria are applied.
Tissue immunophenotyping was performed in 24 cases, 6 of which had frozen tissue. Splenic marginal zone lymphoma with villous lymphocytes shows. 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. 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. 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 slvl responding to 2chlorodeoxyadenosine 2cda. The histopathology of splenic lymphoma with villous lymphocytes. Differential diagnosis of b cell leukaemias with large spleens article pdf available in journal of clinical pathology 406.
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 lymphoma with villous lymphocytes slvl is a lowgrade disorder that regularly presents. Splenic lymphoma with villous lymphocytes slvl in 2005. Rituximab, used alone or in combination, is superior to. Oscier dg, matutes e, gardiner a, glide s, mould s, britobabapulle v, ellis j, catovsky d. Pdf the histopathology of splenic lymphoma with villous.
How i diagnose and treat splenic lymphomas blood american. 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. Splenic lymphoma with villous lymphocytes gupta r, naseem s. Splenic marginal zone lymphoma with villous lymphocytes peripheral blood 3. 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. 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 an overview. Rare disorder, usually considered the leukemic variant of splenic marginal zone b cell lymphoma clinical features. The immunophenotype was consistent with a diagnosis of splenic lymphoma with villous lymphocytes slvl. A case of splenic lymphoma with circulating villous lymphocytes is reported.
Splenic marginal zone lymphoma smzl is a rare form of indolent b. 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. Pathology outlines splenic lymphoma with villous lymphocytes. A type ii igmigg cryoglobulin was detected in the serum. Pdf splenic b cell lymphoma with circulating villous. Splenic lymphoma with villous lymphocytes presenting as. Regression of splenic lymphoma with villous lymphocytes after. 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. The different diagnosis, work up and treatment options are discussed in these guidelines. 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. 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. Splenectomy andor chlorambucil are usually regarded as the most effective treatment in slvl. Splenic lymphoma with villous lymphocytes slvl author. Resembles hairy cell leukemia clinically mean age 72 years, usually males.
Splenic lymphoma with villous lymphocytes slvl is a low. Mar 03, 2011 highgrade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes. Splenic lymphoma with villous lymphocytes semantic scholar. Monoclonal gammopathies in bcell nonhodgkins lymphomas.
Polymorphous population with mantle and marginal zone distribution. Pdf outline for writing an article for current treatment. Extranodal sites and lymph nodes may also be involved. Splenic marginal zone lymphoma an overview sciencedirect. We report a patient with urticaria pigmetosa of ten years evolution and systemic mastocytosis sm diagnosed three years ago who developed splenic marginal zone lymphoma with villous lymphocytes, a lowgrade bcell nonhodgkins lymphoma characterized by splenomegaly, moderate lymphocytosis, bone marrow infiltration, serum monoclonal band. Diagnosis of splenic bcell lymphomas in the bone marrow. Splenic lymphoma with circulating villous lymphocytes. Splenic lymphoma with villous lymphocytes pathology outlines. Pdf the immunophenotype of splenic lymphoma with villous. 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. Splenic lymphoma with circulating villous lymphocytes splenic marginalzone lymphoma.
Bhs guidelines for the treatment of marginal zone lymphomas. Treatment of splenic marginal zone lymphoma with rituximab. The immunophenotype 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. Diagnosis requires either spleen histology or, alternatively, the. Pdf splenic red pulp lymphoma with numerous basophilic. 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.
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 marginal zone lymphoma with villous lymphocytes shows ongoing immunoglobulin gene mutations. Traverseglehen a, baseggio l, bauchu ec, morel d, gazzo s, ffrench m, et al. Cytomorphologic findings can be similar to those of splenic marginal zone lymphoma. 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. Whether this condition is identical to splenic marginal zone lymphoma, or only highly similar, is a matter of debate. We report a case in a 55 year old man with the diagnosis of chronic lymphocytic leukemia. Systemic mastocytosis associated with splenic marginal zone. Presence of disease in peripheral lymph nodes and extranodal locations is uncommon. Splenic lymphoma with villous lymphocytes slvl is a recently recognized entity among chronic b lymphoproliferative disorders.
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. Older names include lymphoma simulating hairy cell leukemia and lymphoplasmacytic lymphoma with circulating villous lymphocytes. Cytogenetic studies in splenic lymphoma with villous lymphocytes. Splenic b cell lymphoma with lymphocyte clusters in. 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. The diagnosis of splenic lymphoma with villous lymphocytes was based on the presence of typical clinical, hematologic, and immunologic findings as previously described. Presenting features and response to therapy have been described, but information on prognostic factors is scanty. The histopathology of splenic lymphoma with villous lymphocytes pg isaacson. 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. Lymphomawith villous lymphocytes phasewas raised by the referring physician. Splenic marginal zone b cell lymphoma stanford university. Highgrade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes. Theclinical and laboratory findings in these patients were compared with those ofseparate groups ofpatients with hcl41 cases, hclvariant five cases, pll 40 cases and cll174cases. A singlecenter experience in splenic diffuse red pulp lymphoma diagnosis.
Renal biopsy demonstrated a membranoproliferative type i glomerulonephritis with intraluminal igg and igm deposits, and a skin biopsy showed a leucocytoclastic vasculitis with. The histopathology of splenic lymphoma with villous lymphocytes article pdf available in blood 8411. The immunophenotype was that of mature b cells without cd5, cd10, cdllc, or cd25 expression or tartrateresistant acid phosphatase. We report the case of a 70 yearold patient with the diagnosis of chronic hepatitis c and splenic lymphoma with villous lymphocytes, who.
We identified lymphoma cases with numerous basophilic vls among the large group. Regression of splenic lymphoma with villous lymphocytes. However, those cells are variably described, and detailed cytologic characterization is often lacking. Splenic marginal zone lymphoma with and without villous. 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. Villous lymphocytes in splenic large bcell lymphoma with. Splenic marginal zone lymphoma with villous lymphocytes peripheral blood 6. Small round lymphocytes surround and replace follicles follicular colonization results in micronodular pattern. Splenic mzl is characterised by micronodular infiltration of the. 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. 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.
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