Colonoscopic red ring sign due to chronic lymphocytic leukemia.
Explore the source record for details and available documents.
Aged↗
Biomedical subjects
D M Menke
Publications and source records attributed to D M Menke.
Mastocytosis: reactive or neoplastic?
Mast cells are now known to derive from CD34+ haemopoietic stem cells in the bone marrow. However, it has not yet been established whether the various types of mastocytosis, which involve tumour-like proliferation of mast cells, are true neoplastic disorders or reactive/hyperplastic conditions. In this study, tissue specimens (five bone marrow, two spleen, one skin) from female patients with histologically confirmed mastocytosis were investigated with a recently developed polymerase chain reaction assay for the determination of clonality of female cells using the human androgen receptor gene (HU-MARA). Mast cells purified to near homogeneity from hysterectomy specimens served as a control. The findings in bone marrow and skin either were not reproducible, or indicated polyclonality. However, both spleen specimens exhibited monoclonality. In addition, DNA analysis by flow cytometry was performed and revealed a diploid chromosome content with proliferation indices of under 8% in all the specimens. This is the first molecular biological study to indicate that mastocytosis is indeed neoplastic in nature.
Case-Control Studies↗
Neoplastic human tissue mast cells express the adhesion molecule CD44/HCAM.
Expression of the homing-associated cell adhesion molecule/HCAM (CD44) in normal/reactive and neoplastic human tissue mast cells (TMC) was determined immunohistochemically using the antibody DAKO-DF1485, which detects all isoforms of CD44. Studies were performed on 30 routinely processed specimens. Twenty of these, from bone marrow, skin, spleen, liver, lymph node and jejunal mucosa, contained infiltrates of TMC. These represented various types of generalized mastocytosis/systemic mast cell disease, including benign systemic mastocytosis. Ten specimens consisted of tissue with a marked reactive increase in TMC; most of these were lymph nodes with chronic nonspecific lymphadenitis and benign or malignant solid tumours. In all 30 specimens TMC exhibited an annular pattern of immunostaining, which was usually very strong. Both normal/reactive and neoplastic TMC exhibited consistent immunoreactivity with the antibody DAKO-DF1485, and this antibody may be of diagnostic value in the detection of atypical TMC associated with malignant mastocytosis. TMC and their neoplastic derivatives belong to a large family of mesenchymal and epithelial cells containing the principal surface receptor for hyaluronan.
Humans↗
Diagnosis of Castleman's disease by identification of an immunophenotypically aberrant population of mantle zone B lymphocytes in paraffin-embedded lymph node biopsies.
Castleman's disease (CD) is characterized by lymph node enlargement due to hyperplasia of abnormal lymphoid follicles and paracortical lymphocytic hyaline vascular (HV) stroma or plasmacytosis (PC). The lymphoid follicles in CD show involuted germinal centers and prominent mantle zone lymphocytes. Ninety-seven cases clinically suspected to be CD were analyzed according to conventional histologic criteria established by Castleman and Keller for diagnosis. Twenty-two cases were excluded as nonspecific hyperplasia (12); Hodgkin's and non-Hodgkin's lymphoma (9); and multiple myeloma involving lymph node paracortex (1). The 75 remaining cases, consisting of 51 cases of CD and 24 with altered follicles or paracortex suggestive of CD, were further analyzed immunohistologically for changes in follicular dendritic reticulum cells (FDRC) using the monoclonal antibody Ki-M4p, for germinal center proliferation with Ki-S5, for mantle zone immunophenotype with Ki-B3 and Ki-B5, for paracortical plasmacytoid monocytes with Ki-M1p, and for plasma cell clonality by applying antibodies to kappa and lambda immunoglobulin light chains. Lymph nodes showing nonspecific follicular and paracortical hyperplasia were included as controls. Hyaline vascular CD and plasma cell CD showed enlarged, polyploid FDRC with prominent nucleoli, decreased germinal center proliferation, and mantle zone populations of immunophenotypically aberrant, Ki-B3-negative B lymphocytes. Thirty-seven percent of hyaline vascular CD and plasma cell CD contained plasmacytoid monocytes, and 15% showed interstitial areas of lambda predominant plasma cells. Plasmacytoid monocytes were common in hyaline vascular CD but rare in plasma cell CD. Cases suspected to be CD that demonstrated a mantle zone population of Ki-B3-negative B lymphocytes had clinical finding of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal paraprotein, and skin changes or sclerotic bone lesions) syndrome and were reclassified as hyaline vascular CD, plasma cell CD, and mantle zone CD with an aberrant mantle zone immunophenotype only (lacking follicular center and paracortical histologic or immunohistologic abnormalities). Immunohistochemistry was valuable for identification of dysplastic FDRC, decreased germinal center proliferation, and plasmacytoid monocytes. In addition, immunohistochemistry was essential for detection of plasma cell clonality, an aberrant mantle zone immunophenotype, and mantle-zone-restricted CD that was devoid of diagnostic alterations of germinal center or paracortex.
Antibodies, Monoclonal↗
Inflammatory pseudotumors of lymph node origin show macrophage- derived spindle cells and lymphocyte-derived cytokine transcripts without evidence of T-cell receptor gene rearrangements. Implications for pathogenesis and classification as an idiopathic retroperitoneal fibrosis-like sclerosing immune reaction.
Sclerosing pseudotumorous immune reactions of the retroperitoneum have been shown to consist of HLA-DR-positive spindle-shaped fibroblasts and macrophages that resemble fibroblasts, and in some instances they contain clonal populations of T lymphocytes not found in granulation tissue, keloids, nodular fasciitis, or fibromatoses. In patients who are iatrogenically immunosuppressed, circulating monocytes may be induced in vitro to transform into spindle-shaped macrophages, and secrete collagen after stimulation by conditioning medium from activated T lymphocytes. The authors investigated a series of five inflammatory pseudotumors (IPT) of lymph node origin for identification of spindle-shaped macrophages, T-cell receptor gene rearrangements, and lymphocyte-derived cytokine mRNA production. All cases of IPT demonstrated spindle-shaped macrophages resembling fibroblasts or myofibroblasts characterized by vimentin, CD45 (LCA), CD68 (KP1) or HAM-56, and HLA-DR(LN3) immunoreactivity and demonstrated production of procollagen-alpha1 (I) mRNA by in situ hybridization. Clonal T-cell receptor chain gene rearrangements were undetectable by polymerase chain reaction. Strong specific lymphocyte-derived interleukin-1beta and interleukin-6 mRNA cytokine transcripts were identified. Although all patients with IPT were managed with steroids and nonsteroidal anti-inflammatory medication, some had treatment-refractory disease. Because all-trans retinoic acid has been demonstrated to inhibit the in vitro transformation of monocytes into collagen-producing spindle-shaped macrophages ("neofibroblasts"), it may be of benefit for patients with IPT.
Antigens, CD↗
[Primary plasmacytoma of the lymph node (plasmocytic lymphoma). The differential diagnosis of nodal plasma-cell proliferates].
HISTORY AND CLINICAL FINDINGS: A 60-year-old man was found on routine examination to have an enlarged, firm, cervical lymph node. He looked older than his age and his general condition was poor. He had no fever, nocturnal sweating or weight loss. Further examination revealed no hepatosplenomegaly on palpation, but numerous enlarged cervical lymph nodes were palpable. INVESTIGATIONS: Histological investigation of a cervical lymph node revealed a marked increase in slightly pleomorphic plasma cells with monotypic expression of IgM-kappa. Multiple myeloma was excluded on the basis of histological and cytological findings in the bone marrow. Serology revealed a mild antibody deficiency syndrome (gamma-globulin 7.8%) with signs of acute inflammation and an increase in alpha 2-globulin. There was no evidence of a monoclonal gammopathy on electrophoresis. A diagnosis of primary nodal plasmacytoma was made. COURSE: As the patient was asymptomatic at diagnosis he was not given chemotherapy. There was no evidence of tumour progression at follow-up examination two months later. His progress will be monitored closely. CONCLUSION: This patient's history is consistent with the prognosis generally associated with primary nodal plasmacytoma that is much better than that of multiple myeloma.
Diagnosis, Differential↗
Hematologic malignancies and the use of methotrexate in rheumatoid arthritis: a retrospective study.
PURPOSE: To evaluate the relationship between use of methotrexate in rheumatoid arthritis patients and development of hematologic malignancies. PATIENTS AND METHODS: We retrospectively analyzed all patients registered at the Mayo Clinic from 1976 through 1992 with rheumatoid arthritis (n = 16,263) cross-indexed with patients registered during the same period with a hematologic malignancy (n = 21,270). Adult patients were selected who had rheumatoid arthritis, were treated with a disease-modifying antirheumatic drug, and subsequently developed a hematologic malignancy. RESULTS: Thirty-nine patients met the selection criteria. Twelve of them had been given methotrexate. The characteristics of those who received methotrexate, including the type of hematologic malignancy, did not differ from those of patients who received other disease-modifying antirheumatic drugs. CONCLUSIONS: Hematologic malignancies are uncommon in patients with rheumatoid arthritis treated with disease-modifying antirheumatic drugs, including methotrexate. There does not appear to be a relationship between the peak or cumulative dose or the duration of methotrexate therapy and the subsequent development of hematologic malignancy. The histologic types of hematologic malignancy seen in the methotrexate-treated patients did not differ from those of patients treated with other disease-modifying antirheumatic drugs.
Adult↗
Primary lymphoma of the spinal cord.
Non-Hodgkin's lymphomas arising in the spinal cord are extremely rare. Only eight single case reports have been well confirmed in the literature. Herein we describe a 59-year-old woman with symptoms attributable to a spinal cord lesion. Physical examination revealed neurologic deficits but no evidence of tumor elsewhere. Although several imaging studies were performed, only magnetic resonance imaging with use of gadolinium revealed the exact site and extent of the lesion. Laminectomy and direct examination of the spinal cord disclosed a discolored region at the level of the 11th thoracic vertebra. A biopsy specimen was obtained, and pathologic examination revealed an intermediate grade, mixed cell lymphoma of T-cell origin. Radiotherapy was administered to the lesion and adjacent region of the spinal cord with use of 6-MV photons and an anteroposterior-posteroanterior technique; the total dose was 45 Gy in 23 fractions. No chemotherapy was given. After 3 years of follow-up, the neurologic signs and symptoms were stable, and repeated magnetic resonance imaging with use of gadolinium showed no residual tumor. In addition to the case report, we review the literature on primary lymphomas of the central nervous system and discuss treatment recommendations.
Female↗
Angiocentric T-cell lymphoma presenting with multiple cranial nerve palsies and retrobulbar optic neuropathy.
Angiocentric T-cell lymphoma (lymphomatoid granulomatosis) may present with prominent central nervous system (CNS) findings with variable radiographic features. We describe a patient who presented with multiple cranial nerve palsies involving the left optic nerve, left facial nerve, left ocular motor nerves, and bilateral acoustic nerves. Enhancement of the right temporal meninges and a cavernous sinus mass were noted on magnetic resonance (MR) scan. A right temporal craniectomy and meningeal biopsy were performed. Meningeal biopsy revealed atypical angiocentric granulomatous lymphoid infiltrates without associated necrosis, giant cells, or granuloma formation. Morphologic and T-cell, receptor gene rearrangement findings were diagnostic of an angiocentric T-cell lymphoma. Retrobulbar optic neuropathy and multiple cranial nerve palsies may be the presenting features of angiocentric T-cell lymphoma. The neurologic and unique radiographic changes in our case expand the previously reported findings in CNS angiocentric T-cell lymphoma.
Aged↗
Bone marrow aspirate immunofluorescent and bone marrow biopsy immunoperoxidase staining of plasma cells in histologically occult plasma cell proliferative marrow disorders.
Immunofluorescent staining (immunofluorescence bone marrow aspirate) and immunoperoxidase staining (immunoperoxidase bone marrow biopsy) were compared in 26 patients with plasma cell dyscrasia and less than 10% marrow plasma cells. Their clinical diagnoses included monoclonal gammopathy of undetermined significance (13 patients), treated multiple myeloma (four patients), multiple myeloma with less than 10% marrow plasma cells (two patients), primary systemic amyloidosis (two patients), monoclonal gammopathy of undetermined significance with neuropathy (two patients), angiofollicular lymph node hyperplasia (two patients, all with the POEMS [polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes] syndrome), and primary (amyloidosis) amyloid neuropathy (one patient). The percentage of plasma cells was greater than 5% in 23% of patients and less than or equal to 5% in 77% of patients. With immunofluorescence bone marrow aspirate and immunoperoxidase bone marrow biopsy, light-chain restriction was demonstrated in 84% of all cases and accurately determined in 96% of all cases as shown by serum and urine paraprotein analysis or tissue amyloid typing. Monoclonal populations of plasma cells can be readily identified with immunofluorescence bone marrow aspirate and immunoperoxidase bone marrow biopsy in most patients with paraproteins and marrow plasmacytoses not diagnostic of multiple myeloma.
Adult↗
Subcutaneous T-cell lymphoma. Report of two additional cases and further observations.
BACKGROUND: Peripheral T-cell lymphomas involving subcutaneous tissue are rare. With modern immunohistochemical stains, subcutaneous T-cell lymphomas may be demonstrated in patients previously thought to have fatal Weber-Christian disease, malignant histiocytosis, and histiocytic cytophagic panniculitis. OBSERVATIONS: We describe two young women with subcutaneous lymphomas. They presented with erythematous nodular lesions, which suggested benign panniculitis. Skin biopsy specimens in both patients demonstrated large atypical lymphocytes in the subcutaneous tissue, karyorrhexis, and cytophagia. Immunohistochemical studies confirmed a T-cell phenotype. Molecular genetics studies performed in one patient showed a major T-cell clonal population. CONCLUSIONS: Subcutaneous T-cell lymphoma is a rare, distinct subtype of postthymic peripheral T-cell lymphoma. Cytophagic "histiocytic" panniculitis is frequently a prominent histologic feature. Early systemic chemotherapy may offer therapeutic advantages in patients with this disease.
Adult↗
Symptomatic gastric amyloidosis in patients with primary systemic amyloidosis.
We reviewed the clinical records of 769 patients with primary systemic amyloidosis who had been examined at Mayo Clinic Jacksonville (Jacksonville, Florida) or Mayo Clinic Rochester (Rochester, Minnesota) during a 12-year period (1978 through 1989). Of these 769 patients, 59 (8%) had biopsy-established gastrointestinal amyloidosis, and 8 (1%) had symptomatic gastric amyloidosis. All eight patients with symptomatic gastric amyloidosis had hematemesis or prolonged nausea and vomiting in association with weight loss. Additional findings were gastroparesis (in three patients), gastric tumor (in one), and gastric outlet obstruction (in one). Macroglossia was present in two patients, and multiple myeloma was diagnosed in three. Six of the eight patients had coexisting small bowel amyloidosis and weight losses of 6.5 to 22.5 kg. Congo red staining identified gastric amyloid in the media of blood vessels in all cases. All cases stained selectively for lambda (seven cases) or kappa (one) light chain. All eight patients died; the median duration of survival after diagnosis was 13.8 months (range, 0.5 to 39.5). Death was due to cardiac failure (three patients), renal failure (two), chronic gastrointestinal obstruction and severe cachexia (two), or hepatic failure (one). Chemotherapy was given to seven patients but was only partially effective for ameliorating symptoms in one.
Amyloidosis↗
Refractory thrombocytopenia. A myelodysplastic syndrome that may mimic immune thrombocytopenic purpura.
The French-American-British classification scheme of myelodysplastic syndromes includes a category of refractory cytopenia that includes refractory thrombocytopenia (RTC). Because dysmegakaryopoiesis manifesting as an isolated cytopenia can be difficult to identify morphologically and because it may be accompanied by megakaryocytic hyperplasia, RTC may be confused with idiopathic thrombocytopenic purpura. A review of 1,220 cases of myelodysplastic syndromes at Mayo Clinic Jacksonville and Mayo Clinic Rochester from 1979 to 1990 yielded 9 cases (0.7%) of isolated thrombocytopenia (RTC) associated with clonal chromosome abnormalities. Review of 319 marrow chromosome analyses performed at the cytogenetics laboratory at Mayo Clinic Rochester from 1979 to 1990 for patients with low platelet count yielded two additional cases of RTC (0.6%). Of the 11 RTC cases, 3 previously had been misdiagnosed as idiopathic thrombocytopenic purpura. All patients had oval macrocytes in peripheral blood smears and abnormal megakaryocyte morphology in bone marrow aspirates, lacked antiplatelet antibodies, and did not have splenomegaly on clinical examination. The most common clonal chromosome abnormalities involved chromosomes 3, 5, 8, or 20. Steroid therapy was ineffective. Clinical and laboratory findings can establish the diagnosis of RTC and allow the physician to avoid recommending inappropriate therapy (steroids or splenectomy) for these patients.
Aged↗
Angiofollicular lymph node hyperplasia: a comparison of unicentric, multicentric, hyaline vascular, and plasma cell types of disease by morphometric and clinical analysis.
A morphometric and clinical analysis of 47 cases of angiofollicular lymph node hyperplasia (AFLH) demonstrated morphometric differences between plasma cell (28 cases) and hyaline vascular (19 cases) AFLH. Twenty-one AFLH cases were unicentric, and 26 cases were multicentric. Plasma cell types of AFLH, defined by standard morphologic and objective morphometric assessment, were associated with systemic clinical and laboratory abnormalities. Unicentric or hyaline vascular AFLH was amenable to surgical therapy, and multicentric or plasma cell AFLH without neuropathy was amenable to steroid treatment or chemotherapy with good prognoses. Multicentric or plasma cell AFLH with neuropathy was associated with resistance to steroids and chemotherapy and with a poor prognosis.
Blood Vessels↗
Isolated polyarteritis nodosa of the male reproductive system.
Polyarteritis nodosa (PAN) frequently affects the testis and epididymis. We describe a 29-year-old man with isolated PAN of the testis and review reported cases of isolated PAN of the testis or epididymis. Our patient underwent orchiectomy and at followup 17 months later was without evidence of recurrence or development of systemic PAN.
Adult↗
Isolated and severe left main coronary atherosclerosis and thrombosis: a complication of acute angle takeoff of the left main coronary artery.
Explore the source record for details and available documents.
Acute Disease↗
Histologic evidence of distal coronary thromboembolism. A complication of acute proximal coronary artery thrombolysis therapy.
Distal migration of occluding coronary thrombus during thrombolysis therapy for acute myocardial infarction rarely has been observed angiographically. Necropsy documentation of the final location of embolic fragments in this circumstance has not been reported previously. This report documents multiple occluded intramyocardial vessels with thrombus observed to have showered distally during thrombolysis therapy. Depending on size and number, fragments of dislodging proximal coronary embolus may produce additional myocardial necrosis.
Adult↗
Hypoplastic coronary arteries and high takeoff position of the right coronary ostium. A fatal combination of congenital coronary artery anomalies in an amateur athlete.
This report describes a previously unrecognized combination of congenital coronary artery abnormalities in the heart of a 30-year-old amateur athlete who died suddenly during a basketball game. Both right and left circumflex coronary arteries were half of their normal length (hypoplastic) decreasing posterior ventricular myocardial perfusion. In addition, the right coronary ostium rose 5 mm above the sinotubular junction (high takeoff position), which also contributed to decreased right coronary artery perfusion. This combination of congenital coronary arterial lesions should be added to the list of structural cardiac defects associated with exercise-related sudden death.
Adult↗