Autoimmune Haemolysis in Childhood and Adolescence

Abstract

The clinico-pathological features of 42 children with autoimmune haemolysis are described. Over 65 % of cases were seen before their 5th birthday. In this group males predominated by the ratio of 2.5:1, but in the older children both sexes were equally affected. The incidence decreased from 1 in 188 × 10³ in young males to 1 in 1,780 × 10³ in children over 10. Cases were classified serologically. Of particular note was the frequency of Donath-Landsteiner haemolysis which equalled that due to warm autoantibodies; together these groups made up 79% of the total cases. Most haemolytic episodes followed an acute infection. This was frequently mild and often involved the upper respiratory tract; in only 2 patients was haemolysis associated with underlying collagenosis. Typically there was a sudden onset of pallor and malaise; jaundice, splenomegaly and hepatomegaly were found in about half the subjects. Haemoglobinuria was characteristic of Donath-Landsteiner haemolysis. The illness was severe, with Hb levels falling below 6.0 g/dl in 28 patients. Prednisolone, blood transfusion and, where indicated, antibiotics were usually effective in treating the illness, with splenectomy reserved for cases where this treatment was unsatisfactory. In several individuals no treatment was required. Recovery was rapid, and complete recovery occurred in 83 % of patients, usually within 6 months. Although 2 patients died, a generally optimistic prognosis can be given, particularly in the absence of an underlying chronic disorder.

New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children

“…2,5,6 Well-defined infection was identified in 22% of our cases but half the cases turned out to be immunological and chronic (Table 4). The main causative agents were Epstein-Barr virus, mycoplasma, parvovirus and cytomegalovirus.…”

“…1 The first descriptions available of this rare disease were provided 40 years ago and were drawn from retrospective series. [2][3][4][5][6] Recent literature contains information from small series, mainly based on laboratory tests, and involving very few centers. [7][8][9][10] The prevalence of AIHA in childhood is still unknown, but likely increases with age, as for most autoimmune disorders.…”

“…[11][12][13][14][15][16] For some authors, infectious causes of AIHA predominate in children. 2,5 For others, most cases of AIHA are primary. 5,6 The underlying pathogenic mechanisms are poorly individualized.…”

“…2,5 For others, most cases of AIHA are primary. 5,6 The underlying pathogenic mechanisms are poorly individualized.…”

Syphilitic paroxysmal cold hemoglobinuria associated with peripheral gangrene: A diagnostic dilemma

“…In the present time, PCH is most often related to infections and neoplasms. Identified pathogens include: measles, mumps, influenza, varicella-zoster virus (VZV), cytomegalovirus (CMV), Epstein-Barr virus (EBV), adenovirus, parvovirus B19, Coxsackie A9, Haemophilus influenzae, Mycoplasma pneumoniae, and Klebsiella pneumonia [4][5][6][7]. Implicated neoplasms include: solid organ malignancy like small-cell lung carcinoma, and hematopoietic disorders such as non-Hodgkins lymphoma (NHL), chronic lymphocytic leukemia (CLL), multiple myeloma and monoclonal gammopathies [8][9][10][11][12].…”