1/148. Ki-1 (CD30)-positive anaplastic large cell lymphoma, sarcomatoid variant accompanied by spontaneously regressing lymphadenopathy.Although it has been reported that primary Ki-1 (CD30)-positive anaplastic large cell lymphoma (ALCL) of the skin may undergo spontaneous regression, it is rare for ALCL without cutaneous involvement to have spontaneously regressing lymphadenopathy. We report a case of sarcomatoid variant of ALCL accompanied by spontaneously regressing lymphadenopathy. The patient had gastric and pulmonary involvement of ALCL in addition to systemic lymphadenopathy, but with no cutaneous involvement. The lymphadenopathy spontaneously improved gradually during a period of one month without any treatment. At the same time, multiple small nodules in both lung fields decreased on chest computed tomography and multiple elevated gastric tumors with dimples were endoscopically recognized to have improved. He has since been treated with combination chemotherapy because of recurrence of the lymphadenopathy.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
2/148. Reversible monoclonal lymphadenopathy in autoimmune lymphoproliferative syndrome with functional FAS (CD95/APO-1) deficiency.The FAS (CD95/APO-1) receptor and its ligand play an important role in the initiation of apoptosis under many physiologic conditions. Loss of function mutations of the FAS gene have been described in lpr mice and in humans with autoimmune phenomena, recurrent lymphadenopathies, and hepatosplenomegaly. This syndrome is now called autoimmune lymphoproliferative syndrome type I (ALPS I). Recently, patients with similar clinical symptoms due to a functional FAS deficiency without FAS gene mutations have been distinguished. This disease has been termed autoimmune lymphoproliferative syndrome type II (ALPS II) or autoimmune lymphoproliferative disease (ALD). This report is the first description of the lymph node pathology and immunohistochemistry in a patient with ALPS II. After recurrent bacterial infections, a 4-year-old child developed cervical giant lymphadenopathy suggesting lymphoma. Lymph node histology resembled the findings in Epstein Barr virus-associated posttransplant atypical lymphoproliferations. Confluent sheets of immunoblasts, however, showed a monoclonal expression of IgG/lambda and a monoclonal rearrangement of the JH chain. The same clone was also present in the peripheral blood. Although high-grade lymphoma could not be excluded, the patient's parents insisted on the patient's leaving the hospital with only antibiotic treatment. Surprisingly, the giant lymphadenopathy completely resolved within 7 weeks, and the clone was no longer detectable in the peripheral blood. Twelve months later the patient was still free from lymphoma and was doing well. Retrospectively, transient monoclonal B-cell populations could be identified in an archival frozen blood sample taken when the patient was 3 years old. Increased FAS-independent spontaneous apoptosis was a feature of the patient's lymphocytes and could be the molecular basis for self-elimination of B-cell clones. We conclude that the diagnosis of a FAS-FAS-L deficiency should be considered in children with an otherwise unexplained atypical lymphoproliferation and that a diagnosis of lymphoma in patients with functional FAS deficiency should be made with considerable reservation.- - - - - - - - - - ranking = 0.8keywords = lymphoma (Clic here for more details about this article) |
3/148. Lymph-node-based malignant lymphoma and reactive lymphadenopathy in eosinophilic fasciitis.BACKGROUND: Lymph node enlargement in patients with eosinophilic fasciitis is a rare occurrence and its clinical significance is unknown. methods: The literature and authors' registries were searched for eosinophilic fasciitis associated with lymphadenopathy. Clinical data, time sequence of appearance of either disorder, and pathological diagnoses were analyzed. RESULTS: Six patients presenting with eosinophilic fasciitis had a lymph-node-based lymphoma and 4 patients had a reactive lymphadenopathy. The patients with lymphoma were elderly and the subcutaneous induration preceded the lymphadenopathy by 2 to 36 months. The patients with eosinophilic fasciitis and reactive lymphadenopathy were young and the onset of subcutaneous induration and lymph node enlargement coincided with one another. Favorable response of the eosinophilic fasciitis to prednisone therapy was attained in 3 of 3 patients with reactive lymphadenopathy and in 4 of the 6 cases with lymphoma. CONCLUSIONS: Eosinophilic fasciitis is rarely associated with clinically significant lymph node enlargement. Subcutaneous induration preceding the lymphadenopathy by 6 months or more, especially in elderly patients, suggests an underlying lymphoma. A favorable response of the subcutaneous induration to prednisone treatment does not exclude the diagnosis of lymphoma; therefore, it does not supersede the need of a pathological evaluation. A lymph node biopsy is mandatory in all cases.- - - - - - - - - - ranking = 1.8keywords = lymphoma (Clic here for more details about this article) |
4/148. Mesenteric lymph node cavitation: a rare hallmark of celiac disease.The cavitation of mesenteric lymph nodes represents a rare complication of celiac disease (only 30 reported cases) whose pathogenesis remains to be clarified. We here report the case of a 67-year-old woman referred to us because of a malabsorption syndrome lasting for 2 years; massive lymph node enlargement and cavitation were detected by means of ultrasonography and a computed tomography scan. celiac disease was definitely diagnosed by means of duodenal histology, and a laparotomy was performed to exclude an underlying T-cell lymphoma. The adoption of a gluten-free diet led to a rapid and dramatic improvement in the clinical and histologic picture and normalization of the size of the lymph nodes. celiac disease should be considered in the differential diagnosis of all patients with mesenteric lymph node cavitation.- - - - - - - - - - ranking = 0.2keywords = lymphoma (Clic here for more details about this article) |
5/148. Thoracic lymphadenopathy in hiv patients: spectrum of disease and differential diagnosis.To evaluate the etiology and differential features of intrathoracic lymphadenopathy (LAD) in hiv patients, chest computed tomography (CT) records from an 18-month period were reviewed to identify all hiv-positive patients with intrathoracic LAD (nodal size > or = 1 cm). medical records were reviewed for the documentation of specific diseases causing LAD and the CD4 count at the time of imaging. Of 45 hiv-positive patients with LAD, 40 had specific diagnoses including 22 (55%) infections and 17 (43%) tumors; one patient had both (3%). Mycobacterial disease accounted for 78% of infections; five cases were secondary to bacterial pneumonia and sepsis. Of tumors, lymphoma (7 cases, 39%) was most common, followed by lung cancer, germ cell tumors, and Kaposi's sarcoma. Mean CD4 cell count in patients with tumors was much higher than in patients with infections (314 vs. 62, p < .01). patients with tumors were somewhat more likely than patients with infections to demonstrate axillary adenopathy (29 vs. 5%, p = .068). Cavitary disease was only observed in patients with infections (27%, p < .03). CT and clinical findings may help direct the differential diagnosis of LAD in AIDS, and promote expedient definitive diagnosis and therapy.- - - - - - - - - - ranking = 0.2keywords = lymphoma (Clic here for more details about this article) |
6/148. Sinus histiocytosis with massive lymphadenopathy and malignant lymphoma involving the same lymph node: a report of four cases and review of the literature.Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a disorder of unknown cause. Rarely, patients with SHML also have malignant lymphoma, usually involving anatomic sites different from those involved by SHML We report four patients in whom SHML and malignant lymphoma were identified in the same lymph node biopsy specimen. The SHML in each case was present as a small focus, less than 1 cm. Immunohistochemical studies showed that the abnormal histiocytes were positive for S-100 and negative for CD1a. The malignant lymphomas included two cases of follicular lymphoma and two cases of Hodgkin's disease, nodular lymphocyte predominant type. The presence of SHML in these patients did not impact clinical decisions, and there was no evidence of SHML elsewhere. Thus, the presence of focal SHML associated with malignant lymphoma in these cases was an incidental histologic finding that seems not to have had any clinical significance.- - - - - - - - - - ranking = 1.8keywords = lymphoma (Clic here for more details about this article) |
7/148. Immunoglobulin related amyloidosis presenting as isolated lymph node and pulmonary involvement.Here we present an unusual case of a 53-year old patient presenting AL-kappa amyloidosis with diffuse-type amyloidosis of lungs, lymph nodes and pleura. The underlying pathology was a B-cell immunoglobulin-secreting non-Hodgkin lymphoma, as proven by the presence of a monoclonal B-cell population in the bone marrow. Diffuse parenchymal infiltration of the lungs is extremely rare in non-systemic amyloidosis, with only 4 previous cases having been reported in the English literature.- - - - - - - - - - ranking = 0.2keywords = lymphoma (Clic here for more details about this article) |
8/148. E rosettes in lymphoproliferative diseases.Results are given of investigations of E rosettes in patients with chronic lymphadenosis and malignant lymphoma. The quantity of E rosettes was expressed both as per cents and in absolute numbers calculated from the total amount of lymphocytes in peripheral blood. In the normal controls the percentage of E rosettes was 58% on average while in patients with chronic lymphadenosis it was found to have decreased significantly down to a mean of 6%. However, in absolute values the number of rosette forming lymphocytes was normal and even higher. In the group with malignant lymphoma the percentage decrease was less striking than in those with chronic lymphadenosis, however, the absolute number of rosette forming lymphocytes was always found to be lowered because of evident to considerable lymphopenia. A dynamic study of the observed changes might contribute to a knowledge of the pathophysiology of lymphoproliferative diseases.- - - - - - - - - - ranking = 0.4keywords = lymphoma (Clic here for more details about this article) |
9/148. An unusual case of pyrexia of unknown origin with cervical lymphadenopathy.Kikuchi's disease is usually a self limiting illness characterised by pyrexia, neutropenia, and cervical lymphadenopathy particularly in young women of Asian descent. This often leads to an initial misdiagnosis of lymphoma. A case of a young Asian woman who presented with pyrexia of unknown origin is described.- - - - - - - - - - ranking = 0.2keywords = lymphoma (Clic here for more details about this article) |
10/148. CD138-positive and Kaposi's sarcoma-associated herpesvirus (KSHV)-negative B-cell lymphoma with serosal spreading of the body cavity and lymphadenopathy: an autopsy case.CD138-positive and Kaposi's sarcoma-associated herpes virus (KSHV)-negative B cell lymphoma with serosal spreading of the body cavity and lymphadenopathy is presented. Our lymphoma cells showed pleomorphic morphology and a clonal immunoglobulin gene rearrangement. Immunophenotypically, they lacked B- and T-cell-associated antigens but expressed strong membranous CD138 antigen along the serosa. Although our case was not conventional primary effusion lymphoma (PEL) because of the absence of KSHV and the presence of lymphadenopathy, its unique phenotype and serosal spreading were consistent with those of PEL. Our case suggests that, irrespective of KSHV infection, some pleomorphic B cell lymphomas with membranous CD138 expression show a peculiar serosal spreading.- - - - - - - - - - ranking = 1.6keywords = lymphoma (Clic here for more details about this article) |
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