Lupus anticoagulant, anticardiolipin antibodies, fetal loss, and systemic lupus erythematosus.
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Antiphospholipid Syndrome↗
Biomedical subjects
D I Feinstein
Publications and source records attributed to D I Feinstein.
Immunoblastic sarcoma: a clinical description.
We reviewed the clinical records of 33 patients with Immunoblastic Sarcoma in order to further describe this disease clinically. Several common features were found. Thirty percent of the patients had a history of a prior immune disease or lymphoproliferative malignancy. Forty-four percent of the patients tested had a diffuse hypergammaglobulinemia. Lymphopenia (less than 1,000/mm3) was found in 45%, and anemia occurred in 73%. At initial presentation, 30% of the cases were clinically staged as either stage I or II, whereas 70% were found to be stage III or IV. Forty-nine percent of the patients had systemic symptoms at presentation. The median survival was 14 months. Advanced stage of disease, lymphopenia, and presence of systemic symptomatology were associated with significantly decreased survival times (p less than .05). We conclude that IBS is a clinical entity often associated with prior immune disease and/or diffuse hypergammaglobulinemia.
Adolescent↗
Use and efficacy of pneumococcal vaccine in patients with Hodgkin disease.
Fulminant bacterial sepsis has been described in patients with Hodgkin disease who have undergone splenectomy for staging purposes. The organisms commonly associated with sepsis in this setting include Streptococcus pneumoniae and Haemophilus influenzae. Polyvalent pneumococcal vaccine (Merck) has recently been licensed and has been suggested for use in patients with Hodgkin disease who are at risk for postsplenectomy sepsis. We administered 14-valent pneumococcal vaccine to 24 patients with Hodgkin disease and 24 normal controls, and measured antibody response to 13 antigens at time of immunization and at 3 wk and 3 mo following immunization. Our results indicate that patients who have been previously treated for Hodgkin disease, with chemotherapy, radiotherapy, or both, have severe impairment of antibody response. Untreated patients, however, respond in a manner similar to normal controls.
Antibodies, Bacterial↗
Quantitative anisocytosis as a discriminant between iron deficiency and thalassemia minor.
The coefficient of variation (CV) of red cell size, as measured by electronic red cell sizing (erythrography), was less than 14.0% in 20 normal subjects. In 22 of 25 patients with beta-thalassemia minor and microcytosis (mean corpuscular volume [MCV] less than 70 fl), CV was less than 14.0%; in the other 3, CV was 14.0%--14.9%. In 53 patients with iron deficiency anemia and MCV less than 70 fl, CV always was greater than 14.0%. In 7 patients with alpha-thalassemia minor and MCV less than 70 fl, CV was less than 14.0% in all 7. Among patients with microcytosis, erythrography appears to be an excellent technique for rapidly distinguishing between iron deficiency and alpha or beta thalassemia minor.
Anemia, Hypochromic↗
Acquired inhibitors of factor V.
The clinical and laboratory data on the 12 patients with an acquired inhibitor to factor V have been reviewed. The degree of clinical bleeding in these patients varied from none to severe, and in most patients the inhibitor was transient. The combination of a markedly prolonged partial thromboplastin time and Quick prothrombin time and failure of normal plasma to correct these tests, in the presence of a normal thrombin and prothrombin and proconvertin time, seems to be pathognomonic for a factor V inhibitor. The inhibitors have physicochemical properties of immunoglobulins and a few have been characterized as polyclonal IgG antibodies or a mixture of IgM and IgG antibodies. The etiology and pathophysiologic mechanism of their development is unknown, but there seems to be a close relationship to major surgery. When tested with inhibitor plasma, the plasmas from 9 patients with hereditary factor V deficiency from 7 unrelated families did not contain factor V antibody-neutralizing material.
Adult↗
Convoluted lymphocytic lymphoma in adults: a clinicopathologic entity.
Twelve adults had a distinct clinicopathologic type of malignant lymphoma that closely resembles the mediastinal lymphomas of childhood. Nine patients presented with mediastinal masses, and seven had symptoms related to intrathoracic compression. Seven patients presented with or developed leukemia, and in four of these patients the central nervous system (CNS) became involved. Structurally, the tumor cells had a distinctive stippled chromatin pattern, in addition to the characteristic nuclear convolutions. Tumor cells from five patients were studied immunologically, and, in each case, the tumor cells formed rosettes with sheep erythrocytes. The response to combination chemotherapy was rapid and dramatic, but usually transient, with relapse in the CNS or previously involved sites. The above data strongly suggest that these cases represent a distinct clinicopathologic entity that should be treated similarly to childhood leukemia and lymphoma, with intensive multiple agent induction, CNS prophylaxis, possibly radiation therapy to initially involved sites, and prolonged maintenance.
Adult↗
Staging laparotomy and splenectomy for Hodgkin's disease.
The major objective of diagnostic laparotomy in Hodgkin's disease is to define the extent of involvement not detectable by nonoperative means. Fifty patients in this institution had operative staging procedures; six for recurrent disease three to 11 years after initial therapy. Twenty-four patients had nodular sclerosis, 23 mixed cellularity, and three had other types. The clinical stages were advanced in 13 patients and decreased in seven patients. Two patients (both had mixed cellularity and systemic symptoms) had positive wedge biopsy of the liver, whereas direct needle biopsy was negative. Nineteen spleens contained Hodgkin's disease but only three could be palpated on physical examination. About half the patients with abnormal lymphangiograms had positive periaortic nodes; lymphangiogram had a false negative rate of 12%. Additional procedures performed included appendectomy, oophoropexy, and resection of Meckel's diverticulum. There was no mortality and only one case had severe postoperative Salmonella septicemia. Our findings are comparable with those reported in the literature.
Adolescent↗
Relationship of contact activation cofactor (CAC) procoagulant activity to kininogen.
Contact activation cofactor (CAC) facilitates the interaction of factors XI and XII. Patients lacking CAC have a coagulation defect and are deficient in high molecular weight kininogen. The coincidence of these two defects suggests that a single protein may be responsible for both physiologic functions. Immunologic and activity studies have been made on isolated CAC to clarify the relationship between CAC and kininogen. CAC forms a single precipitin line with anti-human kininogen, and antikininogen neutralizes CAC activity. CAC and high molecular weight kininogen show a reaction of identity on immunodiffusion against rabbit anti-CAC. Anti-CAC forms two precipitin lines with normal plasma which can be identified as high and low molecular weight kininogen. Monospecific immunoabsorbed anti-CAC forms a single precipitin line with plasma high molecular weight kininogen and neutralizes CAC activity. Cleavage of kinin fragment from CAC by insoluble trypsin or kalikrein does not proportionally reduce procoagulant activity. CAC neutralized by anti-CAC can release kinins on exposure to trypsin or kallikrein. The results support the conclusions that CAC procoagulant activity is a function of high molecular weight kininogen, that antigenic determinants unique to high molecular weight kininogen are shared by the CAC portion of the molecule, and that the clotting reactions may occur at a site removed from the kinin peptide.
Animals↗
Detection of factor VIII inhibitors with the partial thromboplastin time.
Variations of the partial thromboplastin time (PTT) were tested to determine the best screening method for detection of inhibitors of factor VIII. Variables tested included the duration of preincubation of a mixture of patient plasma and factor VIII source (normal plasma), the ratio of the patient plasma to the normal plasma, and the duration of incubation of the normal plasma-patient plasma mixture with kaolin-cephalin suspension prior to recalcification. The following conclusions were reached: (1) The PTT performed on a mixture of equal amounts of patient and normal plasma without preincubation of the mixture was inadequate to detect many factor VIII inhibitors. (2) Factor VIII inhibitors of more than 0.5 Bethesda units could be detected if the PTT was performed on a mixture of four parts patient plasma and one part normal plasma, with preincubation of the mixture for 60 min at 37 degrees C. (3) Factor VIII inhibitors as weak as 0.1 Bethesda units could be detected if the PTT was performed on a mixture of four parts patient plasma and one part normal plasma incubated with kaolin-cephalin suspension for 120 min at 37 degrees C before recalcification. The last method may make detection of mild factor VIII inhibitors possible in routine clinical laboratories not equipped to perform the more technically difficult Bethesda inhibitor assays.
Factor VIII↗
Prothrombin complex concentrate.
A hemophilliac with a factor VIII inhibitor was bleeding massively from an extensive surgical wound. After ten days of unsuccessful management by conservative measures, he was given a single infusion of prothrombin complex concentrate (Konyne). Bleeding stopped immediately and the wound healed without further recurrence of bleeding. Although the activated partial thromboplastin time shortened following the infusion, the exact physiologic mechanism whereby these concentrates bypass the need for factor VIII clotting activity is unknown.
Adult↗
Letter: Rising factor VIII inhibitor titers after Konyne factor IX complex.
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Epitopes↗
Factor XI activity and factor XI antigen in homozygous and heterozygous factor XI deficiency.
A relatively potent antiserum against highly purified, unactivated human factor XI antigen was raised in a rabbit. This antiserum, after concentration, neutralized 50% of the factor XI clotting activity of a standard normal plasma at an antiserum dilution of 1/900. The antiserum was used in a neutralization-inhibition assay to study the relation between factor XI clotting activity and factor XI antigen in plasma from ten unrelated patients with homozygous factor XI deficiency and from 12 heterozygous family members of these patients. No evidence of factor XI antigen significantly in excess of factor XI activity was found in either group. All data to date have been consistent with the hypothesis that hereditary factor XI deficiency represents a genetic disorder resulting from the absence of factor XI molecule. Severity of bleeding in factor XI deficiency could not be correlated with the level of factor XI activity or factor XI antigen.
Animals↗
Determinants of factor VIII recovery in cryoprecipitate.
Many aspects of the production of cryoprecipitate were studied to determine which methods resulted in the greatest recovery of Factor VIII. The following recommendations resulted: 1) blood should be mixed with anticoagulant throughout phlebotomy; 2) blood should be centrifuged within a few hours of collection; 3) larger satellite bags should be used to contain the usual volume of plasma, for example, 200 ml of plasma should be frozen in a 600-ml capacity bag; 4) plasma should be centrifuged as soon as thawing is complete; 5) cryoprecipitate should be refrozen on dry ice; 6) cryoprecipitate should be stored at or below -30 C.; and 7) prolonged storage of frozen plasma or cryoprecipitate should be avoided. Variations in Factor VIII content from one bag of cryoprecipitate to another, under uniform production conditions, depends largely on two donor-specific attributes which tend to remain constant from time to time, namely, the donor's plasma Factor VIII level and the cryoprecipitability of his Factor VIII.
Blood Donors↗