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Biomedical subjects

E S Rappaport

Publications and source records attributed to E S Rappaport.

15 recordsLinked to original sources

Coexistence of Hodgkin's disease and giant lymph node hyperplasia of the plasma-cell type (Castleman's disease).

Coexistence of Hodgkin's disease and giant lymph node hyperplasia (Castleman's disease) is well documented in the literature. We present a unique case in which the original lymph node biopsy revealed interfollicular Hodgkin's disease (CD15+, CD30+, CD45-, Reed-Sternberg cells) with coexistent histologic features of the plasma-cell variant of Castleman's disease. The patient experienced a long-term remission following combined chemotherapy and radiation therapy. He presented at 18 years and again at 22 years later with clinical, hematologic, and histologic features of a multicentric plasma-cell variant of Castleman's disease without evidence of Hodgkin's disease. This unique case report further strengthens the association of Castleman's disease and Hodgkin's lymphoma. Two pathogenetic mechanisms for this association have been suggested: (1) secretion of interleukin-6 by Hodgkin's Reed-Sternberg cells and histiocytes, and (2) manifestation of an abnormal immune state associated with Hodgkin's disease. These two mechanisms may, indeed, be related.

Aged↗

Acute myelomonocytic leukemia in a calf.

In a 2-month-old crossbred calf with paraplegia, results of neurologic evaluation were suggestive of a spinal cord lesion caudal to L3. The calf bled from the blood sampling site for an extended period after venipuncture. Leukocytosis, anemia, and thrombocytopenia were observed. The leukocytes were predominantly atypical blast cells. Postmortem examination revealed petechial hemorrhages throughout the internal organs. Bone marrow was pale tan, with no red marrow seen. Atypical leukocytes were diffusely distributed throughout the body, with penetration of the spinal cord and spinal roots, particularly in the lumbar region. Atypical leukocytes stained positively for alpha-naphthyl acetate esterase and chloracetate esterase, and stained with Sudan black B. Atypical leukocytes expressed class-1 and class-2 major histocompatability antigens, but did not express specific T-, B-, or null-cell surface antigens. The final diagnosis was myelomonocytic leukemia. Differential diagnosis of leukemia in calves should include myelogenous leukemia, and requires use of various techniques to make a definitive diagnosis.

Animals↗

The bone marrow agar section: a morphologic and immunohistochemical evaluation.

A technique for agar embedding of bone marrow aspirate particles is compared with the conventional aspirate smear and bone marrow biopsy by reviewing 503 consecutive bone marrow specimens. Immunohistochemical studies were performed on both agar sections and bone marrow biopsies on 43 paired specimens to compare the results between the two preparations. The results were also compared with traditional clot sections from ten control cases. Of the 382 cases with agar sections, 97.7% contained material in the agar that was diagnostic alone or supportive of the diagnosis made with the biopsy and aspirate smear. In two cases (0.4%), focal involvement by lymphoma was identified on the agar section but not in the biopsy sections or aspirate smears. The immunohistochemical studies showed superior immunoreactivity in agar sections by lymphoproliferative disorders when compared with bone marrow biopsy sections. Similar results between agar and conventional biopsy sections were found in cases of metastatic carcinoma and plasma cell dyscrasias.

Adolescent↗

Complete blood count indices in colorectal carcinoma.

Since carcinomas of the colon or rectum are associated with blood loss, we wondered if complete blood count data were suggestive of iron deficiency in cases of colorectal carcinoma. The mean corpuscular volume and especially the red blood cell distribution width are thought to be more sensitive to early iron deficiency than the hemoglobin value. These values were recorded from a series of 98 consecutive cases of colorectal carcinoma and compared with an age-matched control group consisting of patients with no history or clinical suspicion of malignant neoplasm. We found that the hemoglobin level, mean corpuscular volume, and red blood cell distribution width in patients with colorectal carcinoma do not generally show evidence of iron deficiency. The addition of the mean corpuscular volume and red blood cell distribution width to the hemoglobin value does not seem to increase the sensitivity of the complete blood count in the detection or clinical suspicion of colorectal carcinoma.

Blood Cell Count↗

Colorectal cancer: current trends in initial clinical manifestations.

Colorectal carcinoma (CRC) is a common cause of cancer morbidity and mortality in the United States. There continues to be controversy regarding the effectiveness and feasibility of various screening programs for CRC. To determine how cases of CRC are currently detected, we reviewed a series of 246 consecutive patients with well-documented pathologic staging and clinical presentation. Patients with low stage CRC (0 or A) had smaller tumors, were less likely to be anemic at presentation, and were more likely to have tumors located in the left side of the colon than patients with tumors at stage B or higher. Thirty-two of the 246 tumors were detected in asymptomatic patients through screening. These tumors were more likely to be of a lower stage than those in patients with active gastrointestinal symptoms. In our experience active screening programs detect a relatively small number of CRCs. A majority (66%) of CRCs are still detected from symptoms referable to the gastrointestinal tract.

Aged↗

Angiocentric immunoproliferative lesion (lymphomatoid granulomatosis). A cytogenetic, immunophenotypic, and genotypic study.

We report the occurrence of a cytogenetically abnormal clone 46,XX,t(1;6)(p35;q23),t(1;9;19)(q23;p24;q13) in the spleen of a 23-year-old woman with a three-year history of angiocentric immunoproliferative lesion (AIL) (lymphomatoid granulomatosis). The skin, lungs, spleen, liver and, focally, bone marrow were involved by atypical lymphohistiocytic infiltrates. Immunophenotypic study of the spleen showed that 46% of the cells displayed a helper/inducer T-cell phenotype. However, analysis of DNA isolated from the spleen failed to show clonal T-cell receptor beta-chain gene, T-cell receptor gamma-chain gene, or immunoglobulin heavy chain gene and light chain gene rearrangements. The finding of a cytogenetically abnormal clone supports the concept that angiocentric immunoproliferative lesion is a neoplastic process.

Adult↗

Aplastic crisis in occult hereditary spherocytosis caused by human parvovirus (HPV B19).

We have reported a case of aplastic crisis occurring in an 11-year-old black boy with occult hereditary spherocytosis. An etiologic diagnosis of human parvovirus (HPV) B19 infection was confirmed serologically. The Coulter Model S + IV proved useful for both diagnosis and treatment monitoring through serial histograms. The relationship of HPV infection and aplastic crisis is discussed.

Acute Disease↗

Automated hematology: where we stand.

Rapid, accurate, and relevant laboratory testing is essential in an era of cost-effective medicine. The routine manual complete blood count (CBC) is a labor-intensive test that lacks reproducibility. The new automated instruments such as the Coulter S+ series offer "state of the art" precision and accuracy in counting more than 10,000 cells per blood sample. The nonoptical electronic impedance method yields RBC, WBC, and platelet data, as well as histograms that characterize hematologic data. Analysis of more than 3,500 cases yielded a false-negative rate of less than 4% and a false-positive rate of 30%, indicating the instrument's accuracy in recognizing and flagging abnormalities. All normal screened CBCs were free of significant abnormalities. The Coulter S+ series provides innovative parameters and histograms that aid in establishing differential diagnoses. We review the advantages and the limitations of automation. Morphologic examination of abnormal blood smears remains the cornerstone of diagnosis in both the new and the old hematology.

Anemia↗

Protein C deficiency.

Severe homozygous protein C deficiency is a rare but serious problem in the newborn, with a clinical presentation of purpura fulminans. We have presented such a case in an 1,870 gm female neonate. Salient clinical findings in this case include DIC associated with extensive ecchymosis and subsequent gangrene of the skin, thrombotic complications that began on the third day of life. There was no precipitating infection. The progressive gangrenous necrosis of heel and toes was refractory to heparin therapy, but there was clinical improvement after treatment with fresh frozen plasma. Our patient's level of protein C antigen was less than 3% (normal 70% to 130%). Levels of other vitamin-K-dependent factors, as well as factor V, factor VIIIC, and antithrombin III were normal. A heterozygous protein C deficiency was documented in the mother and father. Presently the child is receiving warfarin sodium (Coumadin) therapy and is clinically stable.

Adolescent↗

Myelodysplastic syndrome: identification in the routine hematology laboratory.

The Coulter S+IV electronically generates an automated white blood cell differential which counts 10,000+ cells per sample, separating lymphocytes, mononuclear cells, and granulocytes. In patients with preleukemic or so-called myelodysplastic syndromes, the histograms are consistently abnormal. CBCs of five patients demonstrate the variable features of myelodysplasia involving abnormal monocytosis, neutropenia, thrombocytopenia, and macro-ovalocytic anemias. The histogram analysis of white blood cells is a rapid, economic way of alerting the hematologist to a possible diagnostic problem in the elderly patient population.

Adult↗

Fatal case of Epstein-Barr virus-induced lymphoproliferative disorder associated with a human immunodeficiency virus infection.

This is a case report of an Epstein-Barr virus-induced polyclonal lymphoproliferative disorder in a presumably immunocompromised patient with Western blot-confirmed antibodies to human immunodeficiency virus. Postmortem examination revealed a diffuse lymphoplasmacytic infiltrate with prominent numbers of immunoblasts involving multiple organs and resulting in multiple organ system failure.

Acquired Immunodeficiency Syndrome↗

A primate model for prolonged endotoxin shock. Blood-vascular reactions and effects of glucocorticoid treatment.

A primate model was developed to study sequential blood-vascular responses, primarily of the lung and liver, and hematologic changes during prolonged endotoxemia with or without glucocorticoid treatment. In this model, pairs of animals, one with intermittent glucocorticoid regimen, were continuously infused with endotoxin throughout the experimental period. The duration of the experiment and the onset of progressive shock could be adjusted by changing the rate of endotoxin infusion. Endotoxemia at a rate of 10 mg. per kg. per hour resulted in progressive shick which was significantly delayed with glucocorticoid treatment. Endotoxin-induced hematologic alterations included early leukopenia and gradual development of disseminated intravascular coagulation. Morphologic studies revealed margination of neutrophils and mononuclear cells in the microcirculation of lung and liver. These changes were associated with sustained phagocytosis of endotoxin by the sequestered leukocytes and Kupffer cells, degranulation of the hepatic sinusoids and spaces of Disse contained extensive fibrinous deposits which in advanced stages of shock were accompanied by midzonal and centrilobular necrosis. Pulmonary lesions included margination, degranulation and fragmentation of leukocytes, early appearance of fibrin in hepatic sinusoids, and rapid development of disseminated intravascular coagulation, endothelial damage and associated lesions of lung and liver. The results indicate that events relating to sustained phagocytosis of endotoxin by the marginating leukocytes initiate a state of intravascular inflammation with disseminated intravascular coagulation and play a vasic role in the pathogenesis of pulmonary and hepatic lesions during prolonged endotoxemia leading to shock. The findings also suggest that glucocorticoid treatment attenuates endotoxin-induced blood-vascular reactions thereby providing an early protection against the development of shock and structural damage to the lung and liver.

Animals↗