1/39. HIV-associated parotid lymphoepithelial cysts.BACKGROUND: An outstanding feature of the diffuse infiltrative lymphocytosis syndrome, or DILS, a subset of hiv-1 disease, is asymptomatic bilateral parotid swelling. Recognition of the entity is important because people with this disease will seek routine dental care. CASE DESCRIPTION: The authors present a classic case of DILS. The patient exhibited bilateral parotid swellings caused by lymphoepithelial cysts, cervical lymphadenopathy, a CD8 circulating lymphocytosis and a CD8 lymphocytic infiltration into the labial salivary glands. A right superficial parotidectomy had been performed several years previously. However, no intervention was advised for the remaining left parotid because of its benign course. CLINICAL IMPLICATIONS: Since patients with DILS can develop lymphomas, periodic observation is mandatory. Any change in the growth pattern requires that a fine-needle aspiration biopsy be performed.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
2/39. Long-term remission in an elderly patient with mantle cell leukemia treated with low-dose cyclophosphamide.We present an elderly patient with mantle cell leukemia who was successfully treated with low-dose cyclophosphamide (CY). A 76-year-old female was diagnosed as mantle cell leukemia based on abnormal lymphocytosis and splenomegaly without lymphadenopathy. She was orally treated with 50 mg of CY daily and had continuous remission over 4 years. Rearrangements of BCL1 and immunoglobulin heavy chain genes in the peripheral blood lymphocytes were detected at diagnosis, but not 1 or 4 years later. Further studies are required to confirm the role of low-dose CY therapy for patients with mantle cell leukemia and lymphoma.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
3/39. Folliculotropic T-cell lymphocytosis (mucin-poor follicular mucinosis).A 48-year-old man presented with multiple asymptomatic patches of hair loss over his trunk and limbs associated with focal keratotic follicular plugs. Multiple skin biopsies showed a panfollicular lymphocytic infiltrate associated with follicular hyperkeratinization, minimal follicular spongiosis, focal basaloid follicular hyperplasia but no overt follicular mucinosis. The lymphocytes were small and there was no atypia. Immunoperoxidase stains showed that the follicular lymphocytes were T cells and predominantly CD4 positive with HLADr (LN3) expressed on their surface. There were insufficient clinical or histopathological features to make a diagnosis of folliculotropic T-cell lymphoma. This case currently may be classified best as folliculotropic T-cell lymphocytosis and may represent a mucin-poor counterpart of follicular mucinosis. Such cases may pursue an indolent course or may evolve to folliculotropic T-cell lymphoma, mycosis fungoides or anaplastic lymphoma. The term folliculotropic T-cell lymphocytosis may be useful for similar cases lacking clinical or histological criteria for lymphoma and lacking follicular mucinosis.- - - - - - - - - - ranking = 4keywords = lymphoma (Clic here for more details about this article) |
4/39. Differentiating hiv-1 parotid cysts from papillary cystadenoma lymphomatosum.BACKGROUND: patients with parotid cystic lesions may first be seen in the dental office. These conditions most often represent either papillary cystadenoma lymphomatosum, or PCL, or lymphoepithelial cysts associated with human immunodeficiency virus, or HIV, disease. The authors present a case report to illustrate the differential diagnosis. CASE DESCRIPTION: PCL represents a benign, usually unilateral, circumscribed parotid tumor with cystic elements. HIV-associated lymphoepithelial cysts of the parotid gland usually are seen bilaterally, create cosmetic concerns and are hallmarked by an associated cervical lymphadenopathy. Therapy for PCL demands surgical excision, while patients with HIV-associated lymphoepithelial cysts may be treated with antiviral therapy and undergo periodic monitoring by a physician. CLINICAL IMPLICATIONS: As a member of the health care team, the dentist must be familiar with head and neck swellings. Early clinical recognition of parotid swellings leads to successful treatment.- - - - - - - - - - ranking = 5keywords = lymphoma (Clic here for more details about this article) |
5/39. The hypereosinophilic syndrome associated with CD4 CD3- helper type 2 (Th2) lymphocytes.We describe herein the clinical and laboratory manifestations of a unique group of patients (pts) presenting with hypereosinophilic syndrome (HES) who were treated in our medical centers for 4-13 years. Skin biopsies, flow cytometry of peripheral blood mononuclear cells (PBMC), assays for cytokines and immunoglobulin (Ig) production in vitro, and Southern blots of T-cell receptor (TCR) genes were performed. All four pts had a persistent hypereosinophilia (> 1.9 x 10(9)/L) and chronic skin rash. Three of four had elevated IgE, thrombotic manifestations and lung involvement (asthma and/or infiltrates), and one had deforming sero-negative arthritis of the hands. 66-95% of their peripheral T-cells expressed CD4 but not CD3 or TCR molecules on the cell surface membrane. Activated CD4 CD3- cells secreted interleukin (IL)-4 and/or 5, and were required for maximal IgE secretion by autologous B-cells. Two pts had evidence of rearrangement of TCR genes of the CD4 CD3- cells, one of whom died of anaplastic lymphoma. In conclusion, HES with CD4 CD3- lymphocytosis may be associated with high serum IgE, dermatological, pulmonary, thrombotic and rheumatic manifestations which may be due to Th2 effects of CD4 CD3- cells migrating to end organs. Fatal systemic lymphoid malignancy may also develop in some pts with monoclonal expansion of the CD4 CD3- T-cells.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
6/39. More than 13 years of hypereosinophila associated with clonal CD3-CD4 lymphocytosis of TH2/TH0 type.A 65-year-old Japanese woman was referred to our hospital because of hypereosinophilia lasting for more than 10 years, and skin ulceration, especially on the hands. Closer examination revealed the clonal proliferation of CD3-CD4 t-lymphocytes. The patient had generalized pruritus without severe end-organ involvement and high serum levels of IgE. A diagnosis of monoclonal CD3-CD4 T-lymphocyte-associated idiopathic hypereosinophilic syndrome (HES) was made based on these findings. This case showed that this newly recognized entity of HES is not restricted to Western countries. The abnormal T-cell clone was not merely TH2 type but was clearly TH2/TH0 type. Although this disease is considered prelymphoma, this patient did not develop lymphoma during more than 13 years of follow-up. Therefore, in some patients, clonal CD3-CD4 lymphocyte-associated HES may take a more indolent course. In this subgroup, the control of clinical manifestations seems very important. In the present case, treatment with hydroxyurea quite dramatically improved the intractable skin manifestations, although the treatment lessened only the number of peripheral eosinophils and not the number of clonal CD3-CD4 t-lymphocytes.- - - - - - - - - - ranking = 2keywords = lymphoma (Clic here for more details about this article) |
7/39. Leptomeningeal involvement in a patient with splenic lymphoma with villous lymphocytes.Splenic lymphoma with villous lymphocytes (SLVL) is an indolent hematological malignancy. Persistent lymphocytosis and splenomegaly usually last for years before patients develop infectious complications. Organ involvement other than spleen and bone marrow is rare in SLVL. We report a case of SLVL-related meningitis occurring in a patient presenting with altered mental status and seizures. CNS involvement was documented by an MRI of the head and by the presence of atypical lymphocytes in the cerebrospinal fluid (CSF). Morphologic examination and immunophenotypic analyses were conducted to determine the nature of atypical lymphocytes in the peripheral blood, spleen, bone marrow and CSF. The patient improved after treatment with a combination of radiation and chemotherapy.- - - - - - - - - - ranking = 5keywords = lymphoma (Clic here for more details about this article) |
8/39. High serum thymus and activation-regulated chemokine levels in the lymphocytic variant of the hypereosinophilic syndrome.The idiopathic hypereosinophilic syndrome is associated with expansion of an IL-5-producing T-cell subset in a subgroup of patients. Identification of such patients is critical to adequate management because there is some evidence that they present an increased risk for development of T-cell lymphoma. Although the T(H)2-like cells often bear an aberrant surface phenotype and can readily be detected with flow cytometry, we now show that lymphocyte phenotyping might be normal in some cases. In contrast, serum thymus and activation-regulated chemokine levels are consistently increased in such patients compared with others with persistent idiopathic hyper-eosinophilia and could therefore represent a useful diagnostic tool.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
9/39. Complete remission of tumour with interleukin 2 therapy in a patient with non-Hodgkin's lymphoma post allogeneic bone marrow transplant associated with polyclonal T-cell bone marrow lymphocytosis.A patient with low-grade non-Hodgkin's lymphoma (NHL) who relapsed shortly after an allogeneic bone marrow transplant (BMT) is reported. The patient was treated with interleukin 2 (IL-2), which resulted in a flare-up of graft-versus-host disease followed by disease control, with disappearance of peripheral lymphadenopathy. Sequential bone marrow testing showed the disappearance of bone marrow involvement with disease but occurrence of T-cell aggregates post IL-2 that were identified as polyclonal by molecular methods. The patient remains in complete remission 37 months following allogeneic BMT.- - - - - - - - - - ranking = 5keywords = lymphoma (Clic here for more details about this article) |
10/39. Dicentric chromosome 3 associated with binucleated lymphocytes in atypical B-cell chronic lymphoproliferative disorder.Binucleated lymphocytes on blood smear are known in PPBL characterized by stable and polyclonal lymphocytosis, polyclonal increase of serum IgM, HLA DR7 and strong correlation with additional i(3q) and premature chromosome condensation. In this disorder some reports of clonal Ig rearrangement suggest a follow up of these patients with immunological and genetic studies. Binucleated lymphocytes are rarely described in other clonal B-CLPD as B-CLL or marginal zone B-cell lymphoma (MZL). Chromosome 3 abnormality is never described in B-CLL but trisomy 3 represents the most consistent abnormality characterizing the MZL. We report in a man without previous medical history an unusual B-CLPD with monoclonal lymphocytosis CD5-, characteristic cytology (particularly binucleated lymphocytes) and chromosomic abnormality as dicentric chromosome 3 never previously described in B-CLPD. In this case lymphocytosis is persistent and stable over 24 months, cytologic immunologic and chromosomic abnormalities are unchanged. We discuss the nosologic place of this atypical B-CLPD closely related to PPBL and MZL with at the moment, after 24 months, a quiet evolution that imply nevertheless a careful follow up with regular cytologic, immunological and genetic studies to clarify the issue.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
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