ACQUIRED HEMOLYTIC ICTERUS

To describe therapeutic plasma exchange as treatment for severe acute hemolytic anemia following human intravenous immunoglobulin (hIVIG) in a dog with primary immune-mediated thrombocytopenia (IMTP). A 4-year-old neutered female Pomeranian with a recent diagnosis of IMTP presented with a potential relapse. Management with initial medications, including prednisolone and mycophenolate mofetil, was unsuccessful; therefore, the dog was administered hIVIG. Fifteen hours later, the patient developed an adverse reaction including vomiting and dark urine. Laboratory and clinical evidence of acute hemolysis was detected, including decreased hematocrit, hemoglobinemia from gross identification of serum, elevated total bilirubin, hemoglobinuria, and reticulocytosis. Supportive care for the dog was ineffective, and due to an adverse reaction characterized by severe salivation, a blood transfusion could not be done. Therapeutic plasma exchange (TPE) was performed to eliminate the causative agents, controlling the hemolytic anemia. After TPE, immunosuppressants were prescribed to treat the IMTP, which resolved 15days later. At the time of this report, the patient is alive with no complications or clinical signs of IMTP. Acute hemolytic anemia associated with hIVIG is a rare adverse reaction in veterinary medicine. This is the first report of TPE being used to successfully treat this potentially life-threatening condition.

In children, paroxysmal cold hemoglobinuria (PCH) is generally considered an acute self-limited autoimmune hemolytic anemia caused by an IgG biphasic auto-anti-P antibody identified by the Donath-Landsteiner (D-L) test. We report a case of a 5-year-old female with a chronic hemolytic anemia. The etiology of the hemolysis appears to be an unusual D-L positive, IgM antibody with specificity for the I antigen. The clinical course is described and a discussion of PCH and the D-L antibody is presented. We also discuss intravenous immunoglobulin infusions as a therapy for children with this form of severe chronic autoimmune hemolytic anemia.

The brown recluse spider (Loxosceles reclusa) is endemic to the southcentral Midwest and the Southern United States. A bite from a brown recluse spider may result in symptoms that range...

Deficiency of the enzyme glucose-6-phosphate dehydrogenase (G6PD) in red blood cells is an inherited abnormality due to mutations of the G6PD gene on the X chromosome that renders the cells vulnerable to oxidative damage. The condition is widespread in many populations living in or originating from tropical and subtropical areas of the world because it confers a selective advantage against Plasmodium falciparum malaria. Clinical features—G6PD deficiency is mostly an asymptomatic trait, but it predisposes to acute haemolytic anaemia in response to exogenous triggers, including (1) ingestion of fava beans—favism; (2) certain bacterial and viral infections; and (3) some drugs—notably some antimalarials (e.g. primaquine), some antibiotics (e.g. sulphanilamide, dapsone, nitrofurantoin), and even aspirin in high doses. Other manifestations include (1) severe neonatal jaundice; and (2) chronic nonspherocytic haemolytic anaemia—the latter is only seen with rare specific genetic variants. The acute haemolytic attack typically starts with malaise, weakness, and abdominal or lumbar pain, followed by the development of jaundice and passage of dark urine (haemoglobinuria). Most episodes resolve spontaneously. Diagnosis relies on the direct demonstration of decreased activity of G6PD in red cells: a variety of screening tests are available, with (ideally) subsequent confirmation by quantitative assay. Prevention is by avoiding exposure to triggering factors of previously screened subjects. Prompt blood transfusion is indicated in severe acute haemolytic anaemia and may be life-saving.

Autoimmune Liver Disease in Three Children with Sickle Cell Disease

SummaryTwo cases of cold hemagglutinin syndrome associated with neurological manifestations were reported. One was a 52‐year‐old woman with acute hemolytic anemia which developed following grippe. Cold hemagglutinin titer was elevated to 1:1,024. Neurological manifestations were apathy, confusion, auditory hallucination and EEG abnormality. Steroid administration cleared these neurological abnormalities and EEG was reverted to normal. The other was a 77‐year‐old man with chronic hemolytic anemia. Cold hemagglutinin titer was extraordinarily elevated (1:32,768, 1:524,‐288). Neurological abnormalities were almost confined to the cerebellar system. Hormonal therapy was completely unrewarding and the patient was incapacitated from his daily activity. From the literature some of the reported cases of hemolytic anemia associated with neurological abnormalities, particularly in reference to cold hemagglutinin syndrome, were briefly described. The present authors consider 2 reported cases fall in the category of “Syndromes neuro‐hémolytiques” by French school.

Infectious Mononucleosis and Acute Hemolytic Anemia; Report of Two Cases and Review of the Literature

Acute Hemolytic Anemia With Acanthocytosis Associated With High-Dose Misoprostol for Medical Abortion

Objective: The aim of the study was to assess the fetomaternal outcome in hemolytic anemia.Method: This study was an observational longitudinal study.Patients with hemolytic anemia were recruited from the antenatal clinic.Routine and special hematological investigations pertaining to hemolytic anemia were done.Blood was transfused with the aim to keep the Hb at 8 gm/dl or above.Maternal and fetal complications were studied.Results: Pallor was the most consistently associated clinical feature in 29 of 37 cases (78%).The most common fetal complication in this study was low birth weight seen in 27 out of 37 cases (73%).Amongst the maternal complications, there were three cases of postpartum hemorrhage and two cases of wound infections. Conclusion:With regular antenatal care, adequate blood transfusion and efficient supervision from the hematology unit, the prognosis of pregnancy in hemolytic disease is not as grave as it was in the past.Expectation to have a family is an important aspiration for a better quality of life for the women with hemolytic anemia.

We have described two clinical cases of severe malaria caused by different pathogens: Pl. falciparum and Pl. malaria, common to which there was a severe course, complicated by acute renal failure and hemolytic anemia. In a detailed analysis of both clinical cases, Patient 1 had acute kidney damage arose after the increase of anemia and thrombocytopenia, in combination with hemoglobinuria. This shows that the leading mechanism of kidney injure in this case is acute tubular necrosis, due to the toxic effects of free hemoglobin and sequestration in the capillaries of the glomerulus. A Patient 2 had a significant increase of anemia after appears of acute kidney damage; there was no hemoglobinuria, however, significant leukocytosis was observed. It seems, that the leading mechanism in this case is immune-mediated kidney injure or due to hypoperfusion of kidney tubules with the development of acute interstitial nephritis or immune complex glomerular injure with the development of glomerulonephritis, or a combination of them. A detailed analysis of the described two clinical cases of severe malaria caused by Pl. falciparum and Pl. malaria, respectively, and complicated by acute renal failure and hemolytic anemia, suggests that the pathogenetic mechanisms and severity of kidney damage depend on the type of malaria.

SUMMARYTwo cases of severe, hemolytic anemia in infants are recorded. The blood findings and the course of the disease differed in several aspects from that of familial, hemolytic icterus. No similar cases were found in the family. Based on these cases and on others reported on in the literature it is suggested that this is a separate disease which must be distinguished from the familial, hemolytic icterus.

In spite of the widespread use of non-steroidal anti-inflammatory drugs (NSAIDs) such as etodolac, induction of drug-dependent antibodies that cause hemolytic anemia and acute tubulointerstitiel nephritis are rarely reported. Currently, there is no way of identifying individuals who are at risk to develop this kind of complications. In patients who present with acute immune hemolysis and acute renal failure, it is important that a careful history of medication be obtained, potential sensitizing medications be identified. In this article we describe a patient with acute autoimmune hemolytic anemia and acute renal failure related with etodolac.

Summary To evaluate the syndrome of acute intravascular hemolytic anemia in black rhinoceroses (Diceros bicornis), the hemoglobin of this species was evaluated by use of isopropanol- and heat-stability tests and was further characterized by electrophoretic studies. Samples were obtained from 22 apparently healthy captive North American black rhinoceroses, though 3 of the study animals had survived previous hemolytic events, and 3 others were parents of 3 offspring that had suffered hemolysis. The eastern African (Diceros bicornis michaeli) and the southern African subspecies (D b minor) were represented. Comparative samples were also obtained from 2 white (Ceratotherium simum) and 1 Indian (Rhinoceros unicornis) rhinoceroses. The hemoglobin of all 3 species appeared stable when tested by use of the heat and isopropanol methods. Thus, an unstable hemoglobin does not appear to be involved in the hemolytic crises of captive black rhinoceroses. Black rhinoceros hemoglobin had a striking polymorphism. Thirteen of the samples from black rhinoceroses had a single hemoglobin band, based on results of alkaline electrophoresis. Nine had, in addition to this major band, a slow (more cathodic) minor band that comprised about 10% of the total hemoglobin. Further studies indicated that the major band and the slower minor band may contain globin chains analogous to human β- and δ-chains respectively; these bands have been tentatively designated B and C. Phenotypes B and BC are common, in a ratio of 4:3. A genetic mechanism is proposed that assumes βb, and βc, gene loci and that βc-locus-expressed (βc+) and βc,-locus-inhibited (βc°) are common alleles for the βc-locus. The polymorphism of rhinoceros hemoglobins appears to be unrelated to the acute hemolytic anemia that occurs in this species.

Acute hemolytic anemia in patients with red blood cells deficient in enzymes such as G-6-PD is characterized by excessive oxidation of hemoglobin (Hb) to a variety of soluble heme pigments and to precipitates, attached to the inner surface of the red cell membrane, known as Heinz bodies (1). These pigments and precipitates do not carry oxygen. The cyanmethemoglobin method. widely used to determine blood Hb concentration, detects not only Hb but methemoglobin and other non-oxygen carrying heme pigments as well; sulfhemoglobin is not detected by this method (2). The present study tested the hypothesis that non-oxygen carrying pigments might constitute a significant fraction of “hemoglobin,” as measured by the cyanmethemoglobin method, during the course of acute hemolytic anemia induced in dogs by phenlyhydrazine, a potent oxidizing agent. Measured blood oxygen carrying capacity (CmaxO2) was less than the CmaxO2 expected from Hb in anemic dogs. The data thus confirm the hypothesis. Additional studies indicat...

Auto immune haemolytic anaemia has been described in association with a variety of hepatotropic viruses, in particular cytomegalovirus, Epstein-Barr virus and hepatitis B. There is a well-recognized association between chronic active hepatitis and auto immune haemolytic anaemia. We present the first reported case of acute hepatitis A which resulted in a fall in haemoglobin concentration from 14.6 to 4.5 g/dl due to an acute haemolytic anaemia with an associated rise in bilirubin from 149 to 960 mumol/l.