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PubMed1990

[Fatal delayed hemolytic transfusion reaction in a postoperative case of traumatic aortic rupture].

A 52-year-old woman with traumatic rupture of the thoracic descending aorta had a history of previous blood transfusion 23 years ago. This time, she received 4,600 ml of blood transfusion during the replacement procedure of thoracic aorta. On the 12th postoperative day, she had acutely progressive severe jaundice, anemia and hepatosplenomegaly. All transfused blood was compatible by bromelin method before operation. Serological studies revealed a secondary response of hemolytic transfusion reaction due to anti E and anti c antibodies. She fell into severe bilirubinemia (66 mg/dl) and anuria, and died on 19th day after operation. A positive Coombs test in a patient who has been transfused recently must be interpreted with great caution. The "coated" cells may be incompatible donor cells in a patient who has antibodies from a prior transfusion. The incompatibility occasionally leads to delayed transfusion reaction that may stimulate "autoimmune" hemolytic anemia.

Anemia, Hemolytic↗
PubMed1990

[Coexistence of myelofibrosis and collagen diseases].

Hematologic abnormalities are common in association with collagen diseases, specially Systemic Lupus Erythematosus and include anemia, neutropenia, thrombocytopenia with alterations in lymphocyte subpopulations. On the other hand, patients with unexplained fibrosis of the bone marrow (the syndrome of idiopathic myelofibrosis or primary myelofibrosis) have clinical and laboratory evidence of immunologic dysfunction. Clinical findings include the presence of arthritis, vasculitis and erythema nodosum. Laboratory abnormalities include the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs test, elevated latex fixation and a circulating lupus type anticoagulant. Total hemolytic complement markedly depressed has also been reported. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis. A review of the literature revealed that myelofibrosis occurring in the setting of collagen diseases is rare. However, a role for immunologic factors in the pathogenesis of myelofibrosis is also supported by the patients with coincident well defined collagen disease and myelofibrosis. In this report, we present two patients with such an association. Case 1 was a 58-year-old male with a two year duration history of rheumatic arthritis. He had bone erosions on hands, splenomegaly and myelofibrosis. Rheumatoid factor (latex) was positive: 1:2560. He had positive LE cells and hypocomplementemia: 37 CH50/ml (NV 70-150). The patient did not meet criteria for SLE. Case 2 was a 36-year-old female admitted because of dyspnea and fever. Diagnosis of myeloid metaplasia with myelofibrosis and progressive systemic sclerosis had been made four years before hand.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1990

[Antiphospholipid syndrome with massive right atrial thrombus. Differential diagnosis of myxoma].

Antiphospholipidsyndrome is a well-defined entity characterized by spontaneous abortion, thrombocytopenia and recurrent arterial and venous thromboses. The diagnosis is biochemically verified by the presence of antibodies binding to negatively charged phospholipids. A patient with recurrent thromboembolic complications over the years eventually developed a rapidly progressing dyspnoe. Two-dimensional echocardiography showed a large right atrial mass which at operation proved to be a thrombus. Coombs test was positive as were lupus anticoagulans and antibodies against cardiolipin, in addition to thrombocytopenia and prolonged bleeding time. The author reviews the literature and discusses problems regarding diagnostical criteria for antiphospholipidsyndrome, pathogenesis of the "procoagulopathy" and thrombembolic complications associated with the syndrome. Special consideration is given to non-invasive diagnostic methods for distinguishing between atrial myxom and floating thrombus.

Aged↗
PubMed1990

Prednisolone and danazol for treatment of immune-mediated anemia, thrombocytopenia, and ineffective erythroid regeneration in a dog.

Immune-mediated anemia and thrombocytopenia were diagnosed in an 8-year-old spayed Miniature Schnauzer. The initial CBC indicated poor erythroid regeneration and concurrent leukopenia. The results of a Coombs test and ANA titer were strongly positive. Examination of a bone marrow specimen revealed erythrophagocytosis and a maturation arrest in the RBC series. Treatment with transfusion plus prednisolone and an attenuated androgen (danazol) resulted in stabilization of the PCV and the development of reticulocytosis.

Anemia, Hemolytic, Autoimmune↗
PubMed1990

[Bestatin-induced intravascular hemolysis in a patient with acute myelocytic leukemia].

We encountered a patient who developed intravascular hemolysis while being treated with immunotherapy of bestatin. A 18-year-old female, diagnosed as having acute myelocytic leukemia and remained in complete remission for about 18 months by repeated consolidation therapy, was admitted to our hospital because of sudden fall of Hb concentration. Nine days prior to admission, bestatin, 30 mg daily, was resumed following a rest period of 2 weeks. Bestatin was started after the first complete remission had been achieved, Urine was dark brown with positive occult blood, but no red cells were observed. Hb was 7.9 g/dl, the number of reticulocytes 14.1%, WBC 3100/microliters with a normal classification, and platelets 6.1 x 10(4)/microliters. The serum total bilirubin was 2.3 mg/dl with a direct component of 0.8 mg/dl. Serum LDH was markedly elevated. Serum Hb was 0.12 g/dl and haptoglobin was less than 10 mg/dl. Direct and indirect Coombs' tests were both negative. Lymphocyte stimulation test by bestatin was positive. Full recovery followed the cessation of bestatin therapy and the initiation of corticosteroid therapy. It may be speculated from our case that discontinuous administration of bestatin may have been an opportune setting for developing a side effect of severe intravascular hemolysis.

Acute Disease↗
PubMedDate not supplied

Acute pancreatitis as the initial manifestation of systemic lupus erythematosus.

A 20-year-old female with three laboratory ARA criteria pro SLE (LE cells, FANA, and positive Coombs test with hemolytic anemia), not under steroid therapy, developed polyarthritis, erythematous rash and acute pancreatitis simultaneously. The latter regressed with high doses of 6-methylprednisolone. Twenty-five months after remission of pancreatitis, no new clinical manifestations (of SLE) have appeared. In another 74 cases of SLE with an average follow-up of 3 years and 8 months there were no other cases of pancreatitis.

Acute Disease↗
PubMed1989

[Fulminant autoimmune hemolytic anemia with multiple organ failure].

We report a rare case of fulminant autoimmune hemolytic anemia (AIHA) with multiple organ failure (MOF). A 40-year-old man was emergently admitted to our hospital because of conscious disturbance and jaundice. The peripheral blood revealed RBC 68 x 10(4)/microliter, Hb 3.5 g/dl, Ht 8.9%, Ret 30% (20,400/microliter), WBC 20,300/microliter, Plts 16.9 x 10(4)/microliter, indirect bilirubin 9.4 mg/dl. Both direct and indirect Coombs test were positive and the IgG autoantibody was identified. Bone marrow aspiration revealed hypercellularity with increased megakaryocytes and erythroid hyperplasia. The patient was diagnosed as having idiopathic warm type of AIHA and the therapy was started with prednisolone 80 mg/day from the first day of admission but hemolysis with reticulocytopenia was so rapidly progressive that he was in acutely life-threatening state and MOF (acute renal failure, adult respiratory distress syndrome, congestive heart failure, liver dysfunction, rhabdomyolysis) appeared on the third hospital day. Plasma exchange therapy and hemodialysis were started and high dose methylprednisolone was given soon after rapid administration of sufficient blood transfusion. Dramatic improvement of hemolysis was noted and MOF was controlled after starting these therapies, but he died of exacerbation of MOF probably due to sepsis 40 days later.

Adult↗
PubMed1989

[Autoimmune hemolytic anemia: clinical analysis of 100 cases].

In 100 cases with autoimmune hemolytic anemia, Coombs test was directly positive in 77 cases (77%). 82 patients had warm autoantibodies. IgM was increased in 12 patients. 3 patients were diagnosed as lymphoma with titers more than 1:1024. One patient had paroxysmal cold hemoglobinuria. The dramatic clinical response to corticosteroid therapy was observed in patients with warm autoantibodies. However, immunosuppressor should be given to patients with cold autoantibodies. It was suggested that combined therapy with two drugs may be more effective than the use of corticosteroid alone. Of 6 patients in whom splenectomy was performed, 4 showed excellent results. Out of 100 cases, there were 23 with Evans syndrome.

Adult↗
PubMed1989

Pulmonary thromboembolism associated with immune-mediated hemolytic anemia in dogs: ten cases (1982-1987).

Pulmonary thromboembolism was confirmed at necropsy in 10 (32.2%) of 31 dogs treated for immune-mediated hemolytic anemia. Radiographic findings associated with thromboembolism included pronounced interstitial lung pattern and small amounts of pleural effusion. Variables associated with significantly higher incidence of pulmonary thromboembolism included hyperbilirubinemia (P = 0.023), negative Coombs test result (P = 0.032), and presence of an indwelling catheter (P = 0.04). There was a tendency (P = 0.06) for association of higher number of whole blood transfusions with pulmonary thromboembolism.

Anemia, Hemolytic, Autoimmune↗
PubMed1989

Hemocytopenia in systemic lupus erythematosus. Relationship to antiphospholipid antibodies.

We studied 500 consecutive patients with systemic lupus erythematosus (SLE) for antibodies to phospholipids (APLA) by an ELISA method using cardiolipin as antigen and antiimmunoglobulins G, M and A to determine their isotype. Once entered into this prospective study the patients were followed for up to 16 months (mean 7.7 +/- 4.72 SD) with periodic determinations of APLA. Of the 500 patients with SLE, 88 had had thrombocytopenia, 25 had had hemolytic anemia, 25 had had both, and 362 had no history of these hemocytopenias. If we considered the odds ratio of these 362 patients for having high titer APLA as 1, patients with a history of thrombocytopenia, hemolytic anemia or both had significantly higher odds ratios of having APLA than did those without hemocytopenia. Patients with thrombocytopenia had significantly higher levels of IgG APLA, those with hemolytic anemia had significantly higher titers of IgM APLA and patients with both had significantly higher titers of both of these APLA isotypes, than did patients without hemocytopenias. A correlation between positive direct Coombs' tests and IgM APLA was also found. We conclude that APLA is associated with these hemocytopenias in SLE. This might be due to their interaction with negatively charged phospholipids in the cell walls of the respective cells.

Anemia, Hemolytic↗
PubMed1989

[Congenital stomatocytosis associated with aplastic anemia].

A seven year-old boy with hereditary stomatocytosis complicated with aplastic anemia was reported. He was admitted to our hospital because of pale and general fatigue. On physical examination, he had severe anemia, petechiae, but no hepatosplenomegaly. Peripheral blood cell count revealed pancytopenia; RBC 103 X 10(4)/microliters, Hb 3.5 g/dl, Ret 21%, WBC 1,200/microliters, Pl 1.3 X 10(4)/microliters, and bone marrow revealed markedly hypocellular marrow. Red cell morphology demonstrated stomatocytosis. Red cell life span (51Cr T1/2) was 12 days, Coombs' test and Ham's test were negative. Indirect bilirubin was 1.1 mg/dl and marked decrease of haptoglobin was found. Family studies showed that his father and sister had stomatocytosis on peripheral blood examination, but no anemia. The patient had severe anemia because of complicated aplastic anemia. Congenital stomatocytosis with aplastic anemia is extremely rare. The authors are interested in a possible relationship between hereditary stomatocytosis and aplastic anemia although the precise mechanism remains to be elucidated.

Anemia, Aplastic↗
PubMed1988

Chromatographically purified vancomycin: therapy of serious infections caused by Staphylococcus aureus and other gram-positive bacteria.

Fourteen patients with serious infections caused by Staphylococcus aureus and other gram-positive bacteria were prospectively treated with chromatographically purified vancomycin in an open-label, nonrandomized study, between December 1986 and June 1987. Five patients were excluded from the evaluation of efficacy. Among the nine evaluable patients, cure was achieved in six patients--a success rate of 67%. One patient had a relapse of osteomyelitis, and cultures of draining pus were positive for oxacillin-resistant S aureus within three weeks after the discontinuation of vancomycin therapy. One patient failed to respond to vancomycin therapy for S aureus-induced endocarditis, meningitis, and osteomyelitis; in another patient, the treatment failed to reverse the course of S aureus septicemia. No serious drug toxicity, for example, nephrotoxicity, was encountered in any patient. One patient (7%) experienced mild ototoxicity. Four patients (29%) had mild phlebitis, two patients (14%) had a transiently positive Coombs' test, and one patient (7%) had a "red neck syndrome" and "pain and spasm syndrome." Chromatographically purified vancomycin is an effective antibiotic in the treatment of serious infections caused by susceptible gram-positive bacteria. Some minor side effects of vancomycin may not be due to impurities in the preparation but rather to the vancomycin itself.

Adolescent↗
PubMed1985

Ceftazidime in the treatment of neonatal infection.

The efficacy of ceftazidime in the treatment of neonatal sepsis was studied in 42 low birthweight premature babies. Forty-nine courses of ceftazidime (25 mg/kg bd, iv or im were administered. In 19 babies, treatment was stopped after 48 h, the remainder were treated for 5 days or more. Six neonates had bacteriological evidence of infection, one other was pyrexial and 29 had radiological evidence compatible with respiratory tract infection. Eight of the study population died. Only one death was attributed to infection which arose 3 days after completion of a 5-day course of ceftazidime. Two babies developed clinical signs of necrotizing enterocolitis (NEC). Clostridium difficile (7) and Cl. perfringens (2) were isolated from 34 post-treatment faecal samples but not from the two babies with NEC. No faecal sample contained Cl. difficile toxin. Post-treatment cultures from 12 neonates yielded ceftazidime-resistant micro-organisms. Ceftazidime therapy was not associated with significant alteration in serum alanine aminotransferase, urea, creatinine, protein or albumin. Four babies had an eosinophilia, three transient and one following two intrauterine transfusions. Coombs' tests were performed on 17 babies. There were no false positives. The abnormal clotting studies observed in one baby were not due to ceftazidime. In a concurrent pharmacokinetic study, the half-life of ceftazidime was 7.4 (SD +/- 4.1) h following iv administration. Other pharmacokinetic values were C max 74 (SD +/- 20) mg l-1 trough concentration 20 (SD +/- 10) mg l-1. Total body clearance ranged from 0.13 to 2.10 ml min-1 per kg and increased with increasing postnatal age.(ABSTRACT TRUNCATED AT 250 WORDS)

Bacteria↗
PubMed1977

Immune haemolytic anaemia after exchange transfusion.

Two cases of immune haemolytic anaemia in newborn infants who had received exchange transfusion on the first day of life because of hyperbilirubinaemia probably due to a conjugation defect, are reported. The first baby, born in the 30th week of gestation presented erythrocyte-fixed IgM antibodies + C, neutropenia, and circulating leucocytotoxic antibodies. The second baby, born at term to a diabetic mother showed erythrocyte-fixed IgG + IgM antibodies + C and a selective IgA deficit. In both cases the anaemia improved and the Coombs test became negative spontaneously in the 4th and 5th months of life, respectively. These haematologic alterations are similar to a graft versus host (GVH) reaction due to the persistence in the neonate blood of the donor's immunocompetent cells.

Anemia, Hemolytic↗
PubMed1978

Autoimmunity and tumor resistance. 3-Methylcholanthrene tumorigenesis in New Zealand Black mice.

Skin tumors induced by the subcutaneous injection of 3-methylcholanthrene (3-MC) in New Zealand Black (NZB) mice had a delayed development and lower frequency compared with BALB/c and C57BL mice. In the SJL/J strain, the incidence of tumors was lower than in the NZB, but with the same delayed development. Most of the tumors in the BALB/c, C57BL, and SJL/J strains were sarcomas; more than one third of the tumors in the NZB mice were squamous cell carcinomas. The greatest frequency and most rapid development of tumors in the NZB, as a function of age at the time of injection of 3-MC, occurred at 4 months. Young (3.5 and 7 weeks) and 12-month-old tumor incidence in the 4-month-old NZB was decreased by treatment with antithymocyte serum (ATS). Five hundred rad whole-body x-irradiation accelerated the onset of tumors but did not increase the final incidence. 3-MC injection and the presence of skin tumors had no influence on the development of glomerulonephritis or hematopoietic neoplasms in the NZB mice. Coombs' positive anemia was not influenced by 3-MC injection, but there was an earlier and increased incidence of positive Coombs' tests in tumor-bearing animals. Liver aryl hydrocarbon hydroxylase (AHH) specific activity was low in the young NZB, increased gradually with age, and was higher in the female mice.

Age Factors↗
PubMed1987

Bone marrow transplantation from an unrelated donor for Fanconi's anaemia: two unusual complications.

An eight-year-old boy with Fanconi's anaemia received a bone marrow transplantation from an unrelated donor. Sustained engraftment was achieved, but he developed major complications including acute grade III graft-versus-host disease, autoimmune haemolytic anaemia with positive Coombs' test and free antibodies against Rhesus group, and a virus-associated haemophagocytic syndrome; this latter complication was successfully treated with acyclovir. Sixteen months after transplantation he has full haematopoietic reconstitution, but with persisting mild autoimmune haemolysis.

Anemia, Aplastic↗
PubMed1988

Thrombocytopenia and hemolytic anemia in a patient with mixed connective tissue disease due to thrombotic thrombocytopenic purpura.

A 33-year-old woman with mixed connective tissue disease (MCTD) presented with headache, fever, thrombocytopenia, hemolytic anemia, and renal involvement due to thrombotic thrombocytopenic purpura (TTP). She did not improve after treatment with prednisolone, fresh frozen plasma, antiplatelet agents, and prostacyclin, but a trial with vincristine resulted in a longlasting complete remission. TTP in autoimmune diseases probably results from immune mediated vasculopathy, which was demonstrated in our patient using nailfold capillary microscopy. Though TTP has many clinical and laboratory features in common with active MCTD, recognition of differences between the 2 conditions, i.e., microangiopathic hemolytic anemia and a negative Coombs' test in the former, is important because treatment in the 2 conditions differs.

Adult↗