Autoimmune hemolytic anemia as an initial presentation in children with systemic lupus erythematosus: two case reports

Abstract

We report the cases of two children who presented with autoimmune hemolytic anemia (AIHA) as an initial presentation of systemic lupus erythematosus (SLE). Both patients had a positive Coombs test, anemia, and an increased number of spherocytes in their blood smear. The patient in Case 1 presented with fever, urticarial erythema, facial paresis, AIHA, and leucopenia. Immunological screening revealed low complement protein levels and positive anti-nuclear antibody, anti-double-stranded DNA, and antiphospholipid antibody results. A further laboratory workup revealed a positive lupus anticoagulant (LA) result and low factor II levels. She was diagnosed with lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) in addition to SLE. The patient in Case 2 presented with fever, butterfly rash, thyroid enlargement, leucopenia, and AIHA. She was diagnosed with SLE with thyroiditis. Both patients were started on combined immunosuppressive therapy, and both patients’ clinical symptoms finally resolved. A literature review on childhood SLE showed that AIHA is common in patients with SLE. LAHPS is an uncommonly identified cause of bleeding in patients with SLE, and it must be considered when evaluating children with a positive LA result.

Lupus Anticoagulant-Hypoprothrombinemia Syndrome: Literature Review and Description of Local Case in a 3-Year-Old Chinese Girl

“…Table 3 presents 33 valid cases with information, excluding 20 cases without a description of the underlying disease. Among these cases, 20 (60.6%) were associated with autoimmune diseases, 2 4 7 9 10 12 14 18 22 24 25 26 27 11 (33.3%) were related to infections, 1 6 11 12 13 16 17 20 21 23 and 2 (6.1%) were linked to tumors. 8 15 The most prevalent autoimmune disease was SLE (14/20, 70.0%), while viruses were the most common etiologic agent of infectious diseases (6/11, 54.5%).…”

“…Out of these, 24 had bleeding at a single site, while 10 had bleeding at multiple sites. The most common bleeding sites were subcutaneous ( n = 16, 47.1%), 2 4 5 7 8 10 12 13 14 16 20 21 27 followed by the urinary system ( n = 9, 26.5%), 5 7 9 10 15 18 27 nose ( n = 8, 23.5%), 2 9 10 11 12 18 intracranial ( n = 6, 17.6%), 9 19 22 23 24 25 gynecological ( n = 4, 11.8%), 7 9 26 gingival ( n = 2, 5.9%), 9 14 and intramuscular ( n = 2, 5.9%). 10 27 Subcutaneous hemorrhage presented as petechiae, ecchymosis, and purpura, while urinary system hemorrhage exhibited gross hematuria, microscopic hematuria, and adrenal hemorrhage.…”

“…The detection rates of LA, aPT, aPS/PT, aCL and aβ2GPI in LAHPS patients were 100% (53/53), 58.3% (14/24), 1,5,15,22,24 50% (12/24), 1,5,6,15,21 47.8% (22/46), 1,2,5,[9][10][11][12][13]15,19,[22][23][24]26,27 and 47.4% (18/38), 1,5,9,11,15,19,[22][23][24]26,27 respectively. There were no significant differences in the detection rates of each of these antiphospholipid antibodies before versus after the age of 18 years (p > 0.05; ►Table 3).…”

“…A total of 27 relevant articles with 53 LAHPS cases were identified as being published in the last 5 years, including our own case. Of these, 13 articles (48.1%) were from Asia, 1 2 4 5 6 7 8 9 10 11 12 13 10 (37.0%) were from Europe, 14 15 16 17 18 19 20 21 22 23 and 4 (14.8%) were from North America. 24 25 26 27…”

“…In contrast to the prothrombotic risk more often associated with LA, patients with LAHPS may experience varying degrees of bleeding, ranging from no bleeding to mild skin and mucosal bleeding and up to life-threatening bleeding. 1 2 The first report of LAHPS was published by Rapaport et al 3 in 1960, describing a severe bleeding case in an 11-year-old girl with systemic lupus erythematosus (SLE) and confirming significantly reduced prothrombin activity. Due to its rarity, there have been limited reports on LAHPS, with less than 200 cases documented in the literature over the past 60 years.…”