Cases reported "Lymphatic Diseases"

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1/27. An unusual case of mediastinal lymphadenopathy caused by amyloidosis.

    A 71-year-old Japanese female Nagasaki-atomic-bomb survivor was admitted for evaluation of a mediastinal mass. She was infected with human T-cell leukemia virus type I. Histological examination of the biopsy specimen, obtained thoracoscopically from the mass, revealed amyloid lymphadenopathy confirmed by congo-red staining and electron-microscopic examination. Amyloid deposits and the serum monoclonal peak consisted of immunoglobulin lambda light chains. No atypical cells were detected in bone marrow. The case was diagnosed as an unusual amyloidosis affecting the mediastinal lymph node. No other sites were found to be affected by amyloidosis, although systemic involvement could not be conclusively ruled out.
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ranking = 1
keywords = leukemia
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2/27. T-cell receptor gammadelta T-cell leukemia with the morphology of T-cell prolymphocytic leukemia and a postthymic immunophenotype.

    T-cell prolymphocytic leukemia (T-PLL) is a postthymic T-cell neoplasm with a characteristic morphology and heterogeneous immunophenotype. Most cases of T-PLL express membrane T-cell receptors (TCRs) of the alphabeta phenotype. We experienced a 30-year-old man suffering from TCRgammadelta T-cell leukemia with morphology compatible to T-PLL with a postthymic phenotype. He was admitted with skin eruption and pancytopenia. Peripheral blood and bone marrow were occupied with medium-sized lymphocytes, which had moderately condensed chromatin with a single nucleolus and sparse, nongranular basophilic cytoplasm. The immunophenotype was CD1a-, CD2-, CD3 , CD4-, CD5 , CD7 , CD8-, and terminal deoxynucleotidyl transferase negative. Hepatosplenomegaly was absent. He was diagnosed as having T-PLL and was treated with combination chemotherapy. Six months later the leukemic cell became chemoresistant. Although the patient showed transient improvement in response to pentostatin, he died 13 months after the diagnosis. To our knowledge, this is the first case of T-PLL with a TCRgammadelta phenotype.
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ranking = 10
keywords = leukemia
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3/27. Nucleolated variant of mantle cell lymphoma with leukemic manifestations mimicking prolymphocytic leukemia.

    Chronic lymphoproliferative disorders sometimes can be difficult to classify. We report 4 cases characterized by large cells with distinct central nucleoli, reminiscent of prolymphocytic leukemia, but shown on further workup to represent mantle cell lymphoma. At initial examination, the patients had generalized lymphadenopathy, splenomegaly, and a leukemic blood picture. The peripheral blood showed many large cells with round to slightly irregular nuclei, single central nucleoli, and a fair amount of pale cytoplasm. The picture was not typical of prolymphocytic leukemia because of the presence of generalized lymphadenopathy and the large size of the circulating abnormal cells. Immunophenotypic study showed that the large lymphoid cells were CD5 CD23- mature B cells with overexpression of cyclin d1, and cytogenetic study demonstrated the translocation t(11;14)(q13;q32) in 3 patients. Lymph node biopsy confirmed a diagnosis of mantle cell lymphoma, pleomorphic variant, in all 4 patients. This study documents the existence of an unusual leukemic form of mantle cell lymphoma with prominent nucleoli; the clinicopathologic features that distinguish it from other chronic lymphoproliferative disorders are discussed.
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ranking = 6
keywords = leukemia
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4/27. Idiopathic myelofibrosis with nodal, serosal and parenchymatous infiltration. Case report and review of the literature.

    Idiopathic myelofibrosis (IMF) is a breakpoint cluster region rearrangement-negative chronic myeloproliferative disease with progressive bone marrow fibrosis. We report a female patient (65 years old) who was admitted to our hospital in 1996. Trephine bone marrow biopsy revealed diffuse fibrosis with atypical multilobulated megakaryocytes. A cellular phase of IMF was diagnosed. Three years later, despite being at intermediate risk, the patient developed generalized lymphadenopathy and multiple sclerosing tumors throughout the peritoneum and retroperitoneum. biopsy specimens from these tumors revealed sclerosing hematopoietic infiltrates. The present case demonstrates that IMF not only can progress to acute leukemia but can also spread uncontrollably, or 'metastasize' with extensive sclerosing hematopoietic tumors throughout the body.
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ranking = 1
keywords = leukemia
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5/27. Fine needle aspiration of squamous cell carcinoma of the skin metastatic to the site of leukemic lymphadenopathy. A case report.

    Metastasis of a cancer to another coexisting tumor is a very rare event. A case of primary squamous cell carcinoma of the skin metastatic to lymph nodes replaced by chronic lymphoid leukemia and diagnosed by fine needle aspiration is presented. To our knowledge, this peculiar case represents the first time that these two concurrent tumors were diagnosed by fine needle aspiration.
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ranking = 1
keywords = leukemia
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6/27. Allergic reaction to chlorambucil in chronic lymphocytic leukemia presenting with fever and lymphadenopathy.

    Chronic lymphocytic leukemia (CLL) is commonly treated with the alkylating agent chlorambucil. Allergic reactions to this chemotherapy are rare. Previous reports include urticaria, angioedema, rashes, toxic epidermal necrolysis, drug fever and one case of immune hemolytic anemia. We report 2 cases that had the identical symptoms of acute onset of high fever and progressive lymphadenopathy. These symptoms disappeared with conservative management. Neither of the patients were treated for infection or disease progression. When the patients were rechallenged with chlorambucil, identical reactions recurred. There was no cross-reactivity with the alkylating agent cyclophosphamide or other types of chemotherapy. This type of reaction to chlorambucil has not been described previously. It is important to recognize this as an allergic reaction. These reactions could be confused with the onset of infection, progression of disease or even a Richter's transformation.
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ranking = 5
keywords = leukemia
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7/27. A case report of Merkel cell carcinoma on chronic lymphocytic leukemia: differential diagnosis of coexisting lymphadenopathy and indications for early aggressive treatment.

    BACKGROUND: Chronic lymphocytic leukemia (CLL) is a monoclonal disorder, characterized by a progressive proliferation of functionally incompetent B lymphocytes. There is increased evidence of association between CLL and skin cancers, including the uncommon Merkel cell carcinoma (MCC). CASE PRESENTATION: A case report of an 84-year old male, who presented with an aggressively recurrent form of MCC on the lower lip, on the background of an 8-year history of untreated CLL. During the recurrences of MCC, coexisting regional lymphadenopathy, posed a problem in the differential diagnosis and treatment of lymph node involvement. Histopathology and immunoistochemistry showed that submandibular lymphadenopathy coexisting with the second recurrence of MCC, was due to B-cell small lymphocytic lymphoma. The subsequent and more aggressive recurrence of the skin tumor had involved the superficial and deep cervical lymph nodes. Surgical excision followed by involved field radiation therapy has been proven effective for both malignancies. CONCLUSION: MCC has a high incidence of regional lymphadenopathy at presentation (12-45%) and even when it arises on the background of chronic leukemia, lymphadenopathy at presentation should be managed agressively with elective lymph node dissection. We overview the postulated correlation between Merkel tumor and CCL, the differential diagnosis of regional lymphadenopathy during the recurrences of the skin tumor and the strategies of treatment.
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ranking = 6
keywords = leukemia
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8/27. syndrome of severe skin disease, eosinophilia, and dermatopathic lymphadenopathy in patients with HTLV-II complicating human immunodeficiency virus infection.

    Two intravenous drug users dually infected with human immunodeficiency virus type 1 (hiv-1) and human T-cell leukemia virus type II (HTLV-II) developed an unusual severe dermatitis characterized by progressive brawny induration, fissuring, and ulceration of the skin, with an associated CD8 cell infiltration in one patient. Both patients had persistent eosinophilia. Lymph node biopsy revealed dermatopathic lymphadenopathy, an unusual pathologic finding in hiv-1 infection but one seen in association with mycosis fungoides and other skin disorders. Two new isolates of HTLV-II virus were established from these patients and were identified as HTLV-II by Southern blotting. This type of skin disease and lymph node pathology has not been found in other intravenous drug users who have been infected with hiv-1 alone or in patients in other risk groups for hiv-1 infection. HTLV-II may play a role in this unique new disease pattern in patients infected with hiv-1.
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ranking = 1
keywords = leukemia
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9/27. The spleen in the spleen syndrome: the association of splenoma with hematopoietic and neoplastic disease--compendium of cases since 1864.

    Splenomas have been described as benign congenital growths. We report six cases of splenomas, all in males. Three had hairy cell leukemia, one had Hodgkin's disease, and one had myeloid metaplasia. Average splenic weight was 1629 gm, and all had multiple splenomas (average diameter 1.89 cm). Splenomas bulged above the cut splenic surface, were pseudoencapsulated, and showed involvement of the underlying disease. A re-evaluation of 105 prior cases are adult (86%; age: 49), with 56% associated with neoplastic disease or hematologic abnormalities. Average spleen weight was 626 gm, and 22% contained multiple splenomas. We conclude that splenomas are most likely an acquired proliferative process strongly associated with neoplastic disease or hematologic abnormalities. Splenomas can also be congenital growths (hamartoma; 26% of the series), which are of little importance.
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ranking = 1
keywords = leukemia
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10/27. Acute leukemia with mediastinal mass, lymphadenopathy, and monocytic precursor cells.

    Cell marker analysis with monoclonal antibodies (MoAb) as well as differentiation studies with the chemical inducer TPA were used to identify a population of apparently lymphoid cells as monocytic precursor cells in a patient with acute leukemia. The initial manifestation of the disease with mediastinal mass and lymphadenopathy was followed by the appearance of small lymphocyte-like blast cells in bone marrow (BM) and peripheral blood (PB). Although a lymph node biopsy revealed an infiltration with monoblasts, the leukemic cells in BM and PB were not classifiable. However, when the patient relapsed after chemotherapy with large monoblasts and again with morphologically lymphoid blast cells, the latter could be classified by treatment with TPA. After incubation with the inducer (12-48 hr) the cells became positive with the MoAB VIM-D5, showed a strong reaction with alpha-naphthyl-acetate-esterase and developed macrophage like morphology, as well as phagocytic properties. During the terminal phase of the disease, small, lymphocyte-like blast cells predominated. These cells could be classified by a panel of MoABs. They expressed myeloid determinants (VIM-D5, VIM-2, MY 9, VIM-12, VIM-13) but showed no reactivity with MoABs specific for lymphocytic cells.
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ranking = 5
keywords = leukemia
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