Systemic lupus erythematosus presenting with mixed‐type fulminant autoimmune hemolytic anemia

Hirano, Yoko; Itonaga, Takaaki; Yasudo, Hiroki; Isojima, Tsuyoshi; Miura, Kenichiro; Harita, Yutaka; Sekiguchi, Mutsuo; Kato, Motohiro; Takita, Junko; Oka, Akira

doi:10.1111/ped.12849

Cited by 9 publications

(5 citation statements)

References 8 publications

(25 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Additionally, her proteinuria level (1,400 mg/gm) raised concern for possible lupus nephritis. AIHA occurs in ~10% of patients with SLE and can be associated with the presence of warm or cold agglutinins (5), or as a mixed type (6), as seen in this patient. Warm agglutinin AIHA is the predominant type seen in SLE, which is mediated by IgG antibodies reacting at body temperature, whereas cold agglutinin AIHA is caused by IgM complement–fixing antibody that reacts at a temperature of 4°C (5).…”

Section: Differential Diagnosismentioning

confidence: 75%

“…The patient discussed in this article had a unique presentation with the presence of concomitant warm and cold autoantibody–mediated hemolytic anemia, or mixed AIHA. Mixed AIHA has been reported in autoimmune disorders such as SLE (6), Sjögren's syndrome (31), and relapsing polychondritis (32) as well as in AITL (33). The older age of the patient and the presence of cold agglutinins raised our concern for a malignant process over SLE and prompted additional evaluation, including the lymph node biopsy, ultimately revealing the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

A 79‐Year‐Old Female Patient With Altered Mental Status and Anemia

Maheswaranathan

Lagoo

Diehl

et al. 2022

Arthritis Care & Research

View full text Add to dashboard Cite

Section: Differential Diagnosismentioning

confidence: 75%

Section: Discussionmentioning

confidence: 99%

A 79‐Year‐Old Female Patient With Altered Mental Status and Anemia

Maheswaranathan

Lagoo

Diehl

et al. 2022

Arthritis Care & Research

View full text Add to dashboard Cite

“…Secondary AIHA is a condition that occurs secondary to inflammatory or autoimmune diseases such as Sjogren's syndrome, scleroderma, dermatomyositis, ulcerative colitis, Crohn'sdisease, and autoimmune thyroid; however it is most commonly seen in SLE (31). In the study from Mayo Clinic and in the largest pediatric study by Aladjiji et al, autoimmune/inflammatory diseases were the most commonly detected secondary diseases in patients diagnosed with AIHA (11,12).…”

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

View full text Add to dashboard Cite

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.

show abstract

“…There have been some reports of adult AIHA secondary to SLE with involvement of not only warm antibodies but also cold antibodies, that is mixed -type AIHA (mixed AIHA). 4 In 5,6 AIHA is often associated with extra hematological features, as sever renal and central nervous system involvement, which may require corticosteroid and immunosuppressive treatment. 7 Glucocorticoids can cause dramatic cessation or marked slowing of haemolysis in about two third of patients.…”

Section: Discussionmentioning

confidence: 99%

“…There have been some reports of adult AIHA secondary to SLE with involvement of not only warm antibodies but also cold antibodies, that is mixed -type AIHA (mixed AIHA). 4 Patient with SLE may develop several haematological complications including anemia, leukopenia, thrombocytopenia. Several case series have shown that autoimmune haemolytic anemia occurs in 10% patients with SLE.…”

Section: Introductionmentioning

confidence: 99%

Auto Immune Mixed Haemolytic anaemia: A rare presentation of SLE

Srikanth¹,

Lokesh²,

Dutta³

et al. 2019

Int J Adv Med

View full text Add to dashboard Cite

Auto Immune Mixed Haemolytic Anaemia (AIHA) is defined as presence of both warm and cold antibodies against patient’s own red blood cells which is diagnosed by monospecific Direct anti-globulin test. Hereby we report a middle-aged women old women who was a known case of hypothyroidism on regular medication, presented with history of fatigability, exercise intolerance and exertional breathlessness of 1 month duration. The patient was subjected for preliminary investigations, which revealed severe anaemia with hemoglobin of 3.6 g% and an increased reticulocyte count of 12% with normal total leukocyte and platelet counts. Peripheral smear revealed anisopoikilocytosis, nucleated RBCs and schistocytes. Biochemical tests for haemolysis was evaluated which showed and elevated LDH levels (780U/L), and a reduced serum haptoglobulin levels. Liver Function test revealed a total bilirubin of 6.8mg/dl with indirect bilirubin of 5.4 mg/dl with normal liver enzymes. Baseline evaluation of Auto immune haemolytic anaemia with coombs test turned out to be positive. Patient was subjected for Mono specific DAT, Indirect Anti-globulin test (IAT) and antibody screening. Mono specific DAT showed both Anti IgG and anti C3 antibodies. IAT test was positive at 4⁰C and negative at 22⁰C and 39⁰C which confirmed that the AIHA was of mixed warm and cold type. On evaluation for connective tissue diseases, patient serum was reactive for ANA and ds-DNA and found to have Systemic Lupus Erythematosus which is a rarity and was responded to corticosteroids.

show abstract

Systemic lupus erythematosus presenting with mixed‐type fulminant autoimmune hemolytic anemia

Cited by 9 publications

References 8 publications

A 79‐Year‐Old Female Patient With Altered Mental Status and Anemia

A 79‐Year‐Old Female Patient With Altered Mental Status and Anemia

The spectrum of underlying diseases in children with autoimmune hemolytic anemia

Auto Immune Mixed Haemolytic anaemia: A rare presentation of SLE

Contact Info

Product

Resources

About