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1/12. Polyclonal systemic immunoblast proliferation: an unusual hematologic entity presenting as a medical examiner case.

    A 43-year-old woman who was receiving oral antibiotics for several days for a superficial foot infection developed a persistent rash, fever, and lymphadenopathy, despite discontinuation of the antibiotic and administration of steroids for a presumed drug reaction. Hours after a subsequent visit to the emergency room for worsening symptoms, she died at home. At autopsy, there was a florid, systemic proliferation of polyclonal plasma cells and immunoblasts infiltrating nearly every organ and tissue of the body, most notably the lymph nodes and spleen. The polyclonal nature of the process was confirmed by immunofixation electrophoresis and immunohistochemistry. Cases of fatal polyclonal systemic immunoblast proliferations are extremely rare, and the trigger for such proliferations is not always known. We review the literature on this unusual entity and discuss the clinical and pathologic findings. ( info)

2/12. Angiocentric immunoproliferative lesions of the lung associated with diffuse renal involvement.

    A 62-year-old Japanese man presented with high fever, cough, and sputa. Computed tomography (CT) scan of the chest revealed lung infiltrates with air bronchogram of the right middle lobe and mediastinal lymphadenopathy. Bronchoscopic examination was performed, and mycobacterium avium complex was detected from bronchoalveolar lavage fluid. Although the administration of clarithromycin and levofloxacin improved the patient's symptoms, the lung infiltrates on chest CT scan gradually worsened. lung biopsy of segments 4 and 8 by video-assisted thorachoscopy revealed angiocentric and angiodestructive massive lymphoplasmocytic infiltrations with multinucleated giant cells, which were compatible with grade II angiocentric immunoproliferative lesions. The patient was found to have deterioration of renal function, and glomerular filtration rate was 32.6 mL/min. His kidneys were enlarged and showed prominent and diffuse uptake of gallium-67 citrate. Percutaneous renal biopsy revealed massive infiltration of CD4 mononuclear cells, plasma cells, and eosinophils in the interstitium and destruction of normal structure of tubules. blood vessels were destroyed and replaced by inflammatory cells. The combination chemotherapy achieved a remission, and the patient has remained free of disease at 2 years after onset of the illness. We recommend the imaging of kidneys for diagnosis and following renal biopsy to evaluate the renal involvement of angiocentric immunoproliferative lesions. ( info)

3/12. Angiocentric immunoproliferative lesion of the lung.

    Angiocentric immunoproliferative lesion (AIL) is the angiocentric and angiodestructive process of lymphoreticular cells with vascular invasion. AIL of the lung is rare. We treated a 57-year-old woman with AIL of the lung in whom chest radiography and computed tomography showed ground-glass opacity in the left lower lobe and lingular segment. Since macroscopical and intraoperative lung biopsy findings could not rule out the possibility of malignancy, including malignant lymphoma, we conducted left pneumonectomy. Immunohistological examination of the tumor showed that infiltrating lymphocytes consistent with AIL. Because tumor markers such as serum LDH and soluble IL-2 receptor increased postoperatively, we conducted systemic chemotherapy, after which elevated serum tumor markers decreased. ( info)

4/12. A case of lymphomatoid granulomatosis/angiocentric immunoproliferative lesion with long clinical course and diffuse brain involvement.

    lymphomatoid granulomatosis (LYG)/angiocentric immunoproliferative lesions (AIL) consist of angiocentric and angiodestructive lymphoreticular proliferation predominantly involving the lungs and other extranodal sites, such as the central nervous system (CNS). This clinical entity is considered as a B cell process related to Epstein-Barr virus (EBV) infection and EBV positive diffuse large B-cell lymphoma. The CNS is involved in 20% of cases of LYG, but initial involvement is rare. In cases without pulmonary symptoms, diagnosis may be difficult. We report a rare case involving initial progression of CNS symptoms followed by a pulmonary abnormality.A 14-year-old girl suffered from high fever, ataxic gait and paraparesis. MRI revealed diffuse T2 high signals with multiple gadolinium enhancements in the cerebellum, brain stem and cerebral white matter. Her symptoms briefly improved after steroid therapy, but ataxia gradually progressed. dyspnea due to pulmonary interstitial involvement appeared when she was 18 years old. Steroid therapy proved effective for respiratory symptoms. At 20 years old she suffered from disseminated intravascular coagulopathy (DIC) and hemophagocytic syndrome (HPS) with respiratory symptoms and repeated seizures. Her symptoms improved after the administration of cyclophosphamide. Mild hemiparesis and gait disturbance appeared when she was 22 years old. MRI revealed new lesions at the basal ganglia and subcortical white matter, brain atrophy and diffuse T2 high intensity of cerebral white matter. cyclophosphamide was effective and there has been no recurrence of symptoms in the last 5 years. We reviewed the non-tumorous LYG/AIL involving the CNS, and discussed the clinical features, MRI imaging and diagnosis of the LYG/AIL. ( info)

5/12. Angiocentric immunoproliferative lesion and angiocentric lymphoma of lymph node in children. A report of two cases.

    AIM: To report two examples of an angiocentric immunoproliferative lesion (AIL) and angiocentric angiodestructive lymphoma (AL) presenting in lymph nodes in children. Most commonly involving extranodal sites, AIL/AL rarely presents in the spleen and lymph nodes. methods/RESULTS: Case 1 presented as a cervical lymphadenopathy in a 3 year old girl being treated for pre-B cell acute lymphoblastic leukaemia. Histological and immunohistochemistry studies revealed an Epstein-Barr virus positive (EBV ), large B cell (CD20 and CD30 ) AIL with large areas of necrosis, the whole resembling lymphomatoid granulomatosis. Case 2 presented as a large supraclavicular lymphadenopathy in a 13 year old boy. histology and immunohistochemistry revealed an EBV-, large T cell (CD45RO, CD56, and CD30 ) AL, presenting the features of so called angiocentric T cell/natural killer cell lymphoma, nasal type. CONCLUSIONS: The term AIL/AL refers to a heterogeneous group of conditions not unique to a particular type of lymphoid cell. These lesions are easily recognised by the histopathologist because of their extremely unusual angiocentric pattern. Although rare, AIL/AL may present as nodal lesions in children ab initio. ( info)

6/12. Angiocentric immunoproliferative lesion of the stomach.

    We report here a rare case of angiocentric immunoproliferative lesion (AIL) of the stomach. The patient was a 61-year-old Japanese female whose medical history was unremarkable. Following a complaint of abdominal discomfort, a submucosal tumour of the stomach was found and gastrectomy was done. Histological examination of the tumour revealed multiple angiocentric or angiodestructive lesions with numerous lymphocytic infiltrates. These vascular lesions were histologically the same as those in benign lymphocytic vasculitis with granulomatosis (BLV) of the respiratory tract. AIL is a distinct entity, including BLV, lymphomatoid granulomatosis and angiocentric lymphoma with BLV representing a good prognosis group of AIL. A survey of the literature suggests that AIL is a spectrum of T-lymphocyte proliferative disorders. To our knowledge, this is the first case of AIL involving the stomach primarily. ( info)

7/12. Virus-associated hemophagocytic syndrome: identification of an immunoproliferative precursor lesion.

    Virus-associated hemophagocytic syndrome (VAHS) is a nonneoplastic, generalized histiocytic proliferation with marked hemophagocytosis associated with a systemic viral infection. Histologic studies of lymph nodes usually show lymphoid depletion and histiocytic proliferation. In this report we describe the case of a patient with Epstein-Barr virus-associated VAHS in which initial lymph node biopsy samples showed an immunoproliferative lesion that preceded the usual generalized histiocytic proliferation. This finding suggests that some cases of VAHS may have an immunoproliferative precursor lesion. ( info)

8/12. Systemic polyclonal immunoblastic proliferations.

    This report describes the clinical and pathologic features of four patients with a florid, systemic immunoblastic proliferation. The blood of these patients exhibited a mild to marked leukocytosis with a high percentage of immunoblasts and plasma cells. The bone marrow also was infiltrated extensively by immunoblasts. Lymph node biopsy specimens from two patients showed near total effacement of the nodal architecture by a diffuse infiltration of immunoblasts and plasma cells. The proliferative process was determined to be polyclonal with immunohistochemical techniques. Cytogenetic studies of bone marrow from two patients showed a pseudodiploid abnormal clone, with a translocation involving a break in band 14q32 in each case. The pathogenesis of these proliferative disorders in unclear, although three patients had some evidence of an acute immune disorder. One of these patients was treated with steroids, vincristine, and cyclophosphamide. Another patient was treated with steroids only, and one patient was treated with steroids and cyclophosphamide. All had rapid regression of the disease process. Two patients are alive and apparently free of disease 31 and 48 months after diagnosis. One died of sepsis. The fourth patient had acquired immune deficiency syndrome (AIDS) and died without therapy. The biology of the immunoblastic proliferation of these patients is uncertain. The immunohistochemical results suggest a reactive, polyclonal proliferation, but the cytogenetic abnormalities in two patients indicate the possibility of a cryptic neoplastic clone. ( info)

9/12. Immunoproliferative small-intestinal disease with lymphoma--diagnostic difficulties and pitfalls. case reports.

    Immunoproliferative small-intestinal disease and related diffuse intestinal lymphoma is a debilitating illness prevalent in south africa. case reports are presented to illustrate that the early course may be deceptively prolonged and 'benign'; diarrhoea is not invariable, and an initial clinical response to antibiotics may occur. The combination of villous atrophy and a predominantly plasma cell infiltration of the lamina propria in jejunal biopsy specimens may indicate lymphoma in adjacent bowel or regional lymph nodes. ( info)

10/12. Spindle cell tumor of lymph node of probable reticulum cell origin associated with multiclonal gammopathy.

    We describe a case of a 48 year old man who presented repeatedly with a neck lymph node tumor. The tumor grew insidiously over 11 years and the patient died at the age of 58 with the generalization of the tumor into the right cervical, paratracheal lymph nodes and spleen. The tumor was composed of spindle shaped cells with occasional intranuclear cytoplasmic pseudoinclusion. At the ultrastructural level the tumor revealed membrane bound crystalline inclusions, which were locally in direct contact with both the nuclear envelope and confronting cisternae of the endoplasmic reticulum. The tumor was associated with multiclonal gammopathy composed of two subsets of IgG/lambda and one subset of IgA/lambda positive paraprotein produced by lymphoid cells surrounding the tumor. We diagnosed the tumor as "spindle cell tumor of lymph node of probable reticulum cell origin associated with multiclonal gammopathy" and consider the lymphoid tissue producing the paraprotein to be reactive. ( info)
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