A case series of pediatric patients with direct antiglobulin test negative autoimmune hemolytic anemia

Miller, Jonathan; Cai, Wei; Andrews, Jennifer; Narla, Anupama

doi:10.1111/trf.15350

Cited by 8 publications

(7 citation statements)

References 9 publications

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“…Different mechanisms are involved in the pathophysiology of hemolysis in the congenital hemolytic anemias (membrane defects, hemoglobin disorders, congenital dyserythropoetic anemia), thrombotic microangiopathies and disseminated intravascular coagulation, which should be considered in differential diagnosis 5 Complete blood count, reticulocyte count, blood smear, DAT, markers of hemolysis (serum lactate dehydrogenase, indirect bilirubin, haptoglobin) and urine dipstick are essential laboratory analyses in suspected AIHA 9 . Diagnosis of PCH is confirmed by positive DAT test and detectable D-L hemolysin, but cases with negative DAT test or false negative D-L test have also been reported 10 …”

Section: Discussionmentioning

confidence: 99%

Severe Acute Kidney Injury in Children as a Rare Complication of Paroxysmal Cold Hemoglobinuria

Zieg,

Flachsová,

Linhartová

et al. 2023

Journal of Pediatric Hematology/Oncology

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Paroxysmal cold hemoglobinuria (PCH) is a rare condition in childhood characterized by complement-mediated premature destruction of red blood cells. PCH is associated with intravascular hemolysis causing hemoglobinuria, which may result in acute kidney injury of various severity. We aimed to retrospectively analyze clinical and laboratory features of children with PCH-associated acute kidney injury received at tertiary Pediatric Hematology and Nephrology Center, University Motol Hospital, Prague, Czech Republic during the period 2016 to 2022. We present here 3 children with PCH-associated acute kidney failure requiring renal replacement therapy. We highlight the association of PCH with kidney disease. Renal parameters and urine examination should be regularly tested in all children with PCH.

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Section: Discussionmentioning

confidence: 99%

Severe Acute Kidney Injury in Children as a Rare Complication of Paroxysmal Cold Hemoglobinuria

Zieg,

Flachsová,

Linhartová

et al. 2023

Journal of Pediatric Hematology/Oncology

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“…This can occur in case of low density of IgG, low avidity of IgG, NK cell-mediated hemolysis and another antibody such as IgA, IgM or a different allotype of IgG which is not detected by polyclonal DCT. [ 8 ] Unless tested further with a high index of clinical suspicion, they can be missed and mis-labeled. In our patient, IgA autoantibody was attached to the red cells in a high number with a strong (4+) reaction on monospecific DCT gel cards, and the patient was managed with a short course of prednisolone.…”

Section: Discussionmentioning

confidence: 99%

“…Unlike established guidelines for the evaluation of AIHA, extensive investigations were not done in most of our patients due to resource constraints. The basic evaluation protocol followed at our center is as follows [ Figure 1 ]:[ 5 6 7 8 9 10 11 12 13 14 15 ]…”

Section: Discussionmentioning

confidence: 99%

Autoimmune hemolytic anemia in children

Arora¹,

Dua²,

Radhakrishnan³

et al. 2021

Asian Journal of Transfusion Science

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BACKGROUND: Autoimmune hemolytic anemias (AIHA) are characterized by the destruction of red cells following the production of autoantibodies directed against them. Although AIHA in children is usually self-limiting, many still succumb to the illness due to delay in the diagnosis and treatment. AIHA in children may be secondary to autoimmune diseases, drugs, or immune deficiencies. Early diagnosis and appropriate immunohematological evaluation can aid in the diagnosis and treatment. OBJECTIVE: To analyze the evaluation, treatment, and outcome of AIHA in children. METHODS: Prospective data of patients aged 0–18 years diagnosed with AIHA between June 2017 and May 2019 were collected. INTERVENTION: Prednisolone was the first-line agent in all; second-line agents included cyclosporine and rituximab. Red cell transfusion was given in those with severe anemia with cardiac decompensation. RESULTS: Eleven patients were diagnosed during the study period. Hemoglobin ranged from 1.2 to 9 g/dl. The initial presentation was severe anemia in 8 children and moderate anemia with thrombocytopenia in 3. The trigger was infection in 5. Polyspecific direct coomb's test (DCT) was positive in 10 patients. 2/10 polyspecific DCT-positive cases on further evaluation had immunoglobulin G (IgG) and C3d positivity, whereas rest 8 had only IgG. One infant was diagnosed with DCT-negative immunoglobulin A-mediated AIHA. 4/11 attained remission following the short course of prednisolone. Cyclosporine was used as the second-line agent in 2 and rituximab was used in 3. Seven children are in sustained remission and off medication. One died within 12 h of diagnosis. CONCLUSION: AIHA is not an uncommon problem in children and can vary in its clinical severity. Early and correct diagnosis helps in deciding appropriate treatment.

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“…and immunodeficiencies (autoimmune lymphoproliferative disease (ALPS), SCID, etc.) (19)(20)(21)(22)(23)(24)(25)(26). DAT may be found as negative in approximately 3-11% of the patients with AIHA.20,25 Patients with negative DAT result, patients who underwent transfusion and patients who underwent a transplantation were excluded.…”

Section: Discussionmentioning

confidence: 99%

The spectrum of underlying diseases in children with autoimmune hemolytic anemia

Özcan

Cansever

Yılmaz

et al. 2021

Journal of Health Sciences and Medicine

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Aim: Autoimmune hemolytic anemia (AIHA) is characterized by the production of antibodies directed against red blood cells. We aimed to describe the clinical presentation, hematologic and biochemical profiles, treatment modalities, underlying diseases and outcomes in children suffering from AIHA. Material and Method: In a retrospectively manner, we consecutively recrutied 62 children (aged 1 month-18 years) with AIHA who had been followed in Erciyes University Child Hospital between January, 2000 and November, 2017. Results: The mean age at time of diagnosis was 61.9±51.9 months (range:1-192) in 62 children including 28 girls and 34 boys. The most common complaints included fatigue and fever while the most common findings were jaundice and hepatosplenomegaly. In 22 children (35.4%), there was a comorbid, active, non-specific infection (upper respiratory tract infection, lower respiratory tract infection, diarrhea, urinary tract infection). At time of diagnosis, mean hemoglobin (Hb) level was 6.9±2.5 g/dL (range: 5-9). The glucocorticoid therapy was given in majority of the patients while no medical treatment was given to 4 patients. Of the patients with primary AIHA, 6 patients were unresponsive to the treatment while one patient responded partially. It was found that there were underlying risk factors in 36 patients with secondary AIHA, as immunodeficiency and autoimmune disorders being the most common risk factors. Conclusion: The immunodeficiencies were highly prevalent in children included. By advance of whole exome sequencing technology, we believe that primary immunodeficiencies was the most common underlying disease in our study detected quite high in presented children.

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A case series of pediatric patients with direct antiglobulin test negative autoimmune hemolytic anemia

Cited by 8 publications

References 9 publications

Severe Acute Kidney Injury in Children as a Rare Complication of Paroxysmal Cold Hemoglobinuria

Severe Acute Kidney Injury in Children as a Rare Complication of Paroxysmal Cold Hemoglobinuria

Autoimmune hemolytic anemia in children

The spectrum of underlying diseases in children with autoimmune hemolytic anemia

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