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Biomedical subjects

J P Abraham

Publications and source records attributed to J P Abraham.

16 recordsLinked to original sources
PubMed1989

Myelodysplasia with bone marrow lymphocytosis and fibrosis mimicking recurrent Hodgkin's disease.

Myelodysplastic syndromes and acute leukemias after treatment for Hodgkin's disease (HD) are well recognized. Preleukemic changes are commonly seen. Three patients from the authors' files are found with myelofibrosis and bone marrow lymphocytosis after treatment for HD. Although somewhat unusual, the clinical impression initially was, in each case, isolated recurrence of HD involving the bone marrow, 6 to 11 years after initial diagnosis. In each case, after single or multiple marrow aspirates and biopsies were done, the differential between HD involving the marrow and myelodysplasia with myelofibrosis and lymphocytic infiltrates was made. The absence of diagnostic Reed-Sternberg (RS) cells was useful in diagnosing the latter. All three patients died of causes related to cytopenias, with two having progressed to overt acute nonlymphocytic leukemia. Myelofibrosis with lymphocytic infiltrates in the marrow, without diagnostic RS cells, in patients' status after treatment for HD, should alert one to the possibility of myelodysplasia.

Adult↗
PubMed1989

Platelet function and structure in myeloproliferative disease, myelodysplastic syndrome, and secondary thrombocytosis.

Platelet function and morphologic characteristics were evaluated in 43 patients with myeloproliferative disease (MPD), 5 patients with myelodysplastic syndrome (MDS), and 7 patients with secondary thrombocytosis (ST). Platelet Factor IV (PF4) and B-thromboglobulin (BTG) showed slight elevation in ST but significant elevation in all MPDs. They were either normal or slightly elevated in MDS. Defective platelet aggregation with one or more inducers was seen in 62% of all patients. An epinephrine-induced defect was the most consistent aggregation abnormality. Hyperaggregation and spontaneous aggregation were seen in 15% of patients. Of the eight patients who showed increased bleeding tendency, all eight showed defective aggregation with two or more inducers, five showed decreased surface activation response, as well as decreased or abnormal granules and dense tubular disarray in the transmission electron microscope (TEM) study. Seven patients had clinical evidence of recurrent arterial and venous thromboses. Five of these patients showed hyperaggregation response to adenosine diphosphate and collagen and abnormal Wu and Hoak platelet aggregate ratio. Four patients showed spontaneous aggregation on aggregometer. Surface activation response was significantly increased in five patients, and an increase in platelet granules by TEM study was seen in four patients. Primary thrombocythemia could be differentiated from secondary thrombocytosis (ST) by the presence of abnormal aggregation response and significantly increased PF4 and BTG in the former, and greatly elevated plasma fibrinogen and Factor VIII, as part of acute phase reactant response, in the latter.

Aged↗
PubMed1986

Nonsecretory multiple myeloma with osteoporosis: immunocytologic and bone resorptive studies.

Two patients, ultimately found to have advanced nonsecretory multiple myeloma, presented with skeletal pain, diffuse skeletal demineralization, and fractures. The correct diagnosis was initially obscured by the absence of typical hematologic findings and discrete lytic bone lesions. Bone marrow examination was diagnostic. Intracytoplasmic IgA or IgD kappa was demonstrated in the myeloma cells of each case. Decreased quantitative polyclonal serum immunoglobulins and hypercalcemia were important clinical clues. The demonstration of increased osteoclast activating factor (OAF) derived from the cultured myeloma cells from each case suggests that the secretion of OAF and immunoglobulin are unrelated.

Bone Resorption↗
PubMed1986

Splenectomy in systemic lupus erythematosis.

Of 860 patients with systemic lupus erythematosus (SLE) who were evaluated during a 25-year-period, 16 (1.9%) underwent splenectomy. Twelve of these patients had steroid resistant thrombocytopenia. An excellent long-term outcome occurred in eight (67%), significant improvement occurred in three (25%), and one patient died who also had chronic active hepatitis and portal hypertension. In two of three patients (67%) with autoimmune hemolytic anemia, the condition was corrected by splenectomy; in the third patient there was some improvement, but reduced doses of corticosteroids were required. One patient with severe neutropenia and recurrent bacterial infection obtained lasting benefit following splenectomy. Histologic examination of the removed spleen was not helpful in corroborating the diagnosis of SLE in these well established cases. Splenectomy had no adverse affect upon other aspects of SLE, in particular upon renal function. The authors conclude that the indications for splenectomy have proven to be of value in selected SLE patients with autoimmune or hypersplenic cytopenia.

Adolescent↗
PubMed1984

High incidence of monoclonal proteins in the serum and urine of chronic lymphocytic leukemia patients.

Chronic lymphocytic leukemia (CLL) is generally considered a nonsecretory B cell immunoproliferative disorder. Conventional electrophoretic and immunoelectrophoretic methods have revealed serum monoclonal proteins in less than 10% of these patients. However, there is increasing experimental evidence from in vitro studies demonstrating that CLL cells may secrete immunoglobulins, particularly free light chains. We examined the serum and urine of 36 consecutive CLL patients for monoclonal proteins using sensitive immunochemical methods (high resolution agarose gel electrophoresis combined with immunofixation). The results obtained were correlated with the Rai stage, quantitative immunoglobulin levels, and lymphocyte membrane immunoglobulin phenotype of the leukemic cells. Twenty-three monoclonal proteins were identified in the serum or urine of 22 patients, an incidence of 61%. Six patients had serum monoclonal proteins, seven had only urinary monoclonal proteins, and nine had monoclonal proteins in serum and urine. In every instance the monoclonal protein was the same light chain type as expressed on the leukemic cells. Our findings suggest that the monoclonal proteins observed in the serum or urine of CLL patients are secretory products of the tumor cells and that their discovery is a function of the sensitivity of the method used for their detection.

B-Lymphocytes↗
PubMed1981

Multiple active X chromosomes in myelofibrosis with myeloid metaplasia.

A woman with myelofibrosis and myeloid metaplasia had a karyotype of 47,X,del(X)(q22),+del(X)(q22) in unstimulated peripheral blood and bone marrow aspirate cultures. The normal X chromosome was late replicating, and the two deleted X chromosomes always replicated early and synchronously. The karyotype from phytohemagglutin-stimulated peripheral blood cultures was uniformly 46,XX. Structurally abnormal X chromosomes are exceedingly rare in myeloproliferative disease. The abnormal karyotype very likely reflects monoclonal proliferation of an abnormal myeloid cell line. The X chromosome inactivation process, which acts upon embryonic somatic cells of all mammals, apparently does not react to postembryonic nondisjunction of the active X chromosome.

Bone Marrow↗
PubMed1975

Fitzgerald factor: a hitherto unrecognised coagulation factor.

A newly recognised asymptomatic coagulation-factor deficiency is reported in a 71-year-old man. This factor seems to operate at an early stage in the intrinsic coagulation pathway and participates in other Hageman-factor-mediated biological reactions. The patient has an atypical cellular inflammatory response as observed by the skin-window technique.

Aged↗
PubMed1975

Fitzgerald Trait: Deficiency of a Hitherto Unrecognized Agent, Fitzgerald Factor, Participating in Surface-Mediated Reactions of Clotting, Fibrinolysis, Generation of Kinins, and the Property of Diluted Plasma Enhancing Vascular Permeability (PF/Dil).

The prolonged partial thromboplastin time observed in the plasma of a 71-yr-old asymptomatic man was related to the deficiency of a hitherto unrecognized agent. The patient's plasma also exhibited impaired surface-mediated fibrinolysis and esterolytic activity and impaired generation of kinins and of the property enhancing vascular permeability designated PF/Dil. The patient's plasma contained normal amounts of all known clotting factors except Fletcher factor (a plasma prekallikrein) which was present at a concentration of 10-15% of pooled normal plasma. Fletcher trait plasma, however, contained normal amounts of the agent missing from the patient's plasma and corrected the defects in clotting, fibrinolysis, and vascular permeability. Fletcher trait plasma was less effective in correcting generation of kinins and esterolytic activity, presumably because of the patient's partial deficiency of prekallikrein. The site of action of the factor deficient in the patient's plasma appeared to be subsequent to the activation of Hageman factor and plasma prekallikrein. A fraction of normal plasma, devoid of other clotting factors, corrected the defect in clotting in the patient's plasma; a similar fraction of the patient's plasma did not correct this abnormality. No evidence yet exists pointing to the familial nature of the patient's defect. Tentatively, the patient's disorder may be referred to by his surname as Fitzgerald trait, and the agent apparently deficient in his plasma as Fitzgerald factor.

Journal Article↗