Standard-dose intravenous anti-D immunoglobulin versus intravenous immunoglobulin in the treatment of newly diagnosed childhood primary immune thrombocytopenia.

BACKGROUND: We conducted a study to evaluate the efficacy of intravenous (IV) anti-D against IV immunoglobulin (IVIG) in newly diagnosed immune thrombocytopenia (ITP) in children and to identify the clinical characteristics of the children most likely to benefit from one or the other treatment. PROCEDURE: Children (6 mo to 14 y) with newly diagnosed ITP and a platelet count <20,000/µL were treated either with a single bolus dose of 50 µg/kg IV anti-D or with 0.8 to 1 g/kg IVIG in a randomized manner. RESULTS: Twenty-five patients, mean age of 6.8 years, were treated either with IV anti-D (n=10) or with IVIG (n=15). Both drugs were equally efficient in raising the platelet count above 20,000/µL at 24 hours posttreatment. Children who presented with bleeding stage 1 or 2 (no mucosal bleeding) responded better to IVIG treatment, in terms of an increase in platelet count at 24 hours posttreatment (P=0.04). Hemoglobin drop was greater in the anti-D group (P=0.002). CONCLUSIONS: A single bolus dose of 50 µg/kg of IV anti-D is a safe and effective first-line treatment in newly diagnosed ITP in childhood and mucosal bleeding is a poor prognostic factor for treatment with IVIG.

Hemolytic anemia presenting with idiopathic intracranial hypertension.

We report on an 8-year-old girl with hemolytic anemia because of infection with parvovirus B19 and increased intracranial pressure. She presented acutely with headache, vomiting, and mild scleral and mucosal icterus. Upon evaluation, the patient exhibited profound hemolytic anemia, papilledema, and increased intracranial pressure. The patient was treated with intravenous immunoglobulin, prednisone, and packed red blood cells. Concurrent with an improvement of her anemia, she experienced a gradual resolution of her headache, vomiting, and optic-disc swelling. Signs of idiopathic intracranial hypertension may occur as a consequence of severe anemia, and are reversible upon correction of the underlying hematologic disorder.

The effect of dietary habits and socioeconomic status on the prevalence of iron deficiency in children of northern Greece.

BACKGROUND: Despite encouraging reports concerning the declining prevalence of iron deficiency, this easily preventable disorder is still an existing problem in presumably developed regions. OBJECTIVE: To evaluate the prevalence of iron deficiency and relevant anemia in children residing in Northern Greece and to study possible associations. DESIGN: 3,100 children aged 8 months to 15 years were evaluated. Socioeconomic status was determined based on the parents' profession and place of residence. Nutrition habits were also evaluated. RESULTS: The incidence of iron deficiency was found to be 14% and that of iron deficiency anemia was 2.9%, with a higher prevalence in children younger than 2 years of age. The place of residence was the most significant factor in relation to the development of iron depletion in the children studied. Additional independent factors were revealed to be breast-feeding, meat-containing meal consumption and the consumption of non-home-cooked meals. CONCLUSION: Iron deficiency remains prevalent in Northern Greece, mainly affecting the vulnerable toddler group. Nutritional iron deficiency is still a severe public health problem even in what are considered to be developed regions. An improvement of dietary habits and an upgrading of semiurban areas should contribute substantially to decreasing the prevalence of iron depletion in Greek children.

Rare thalassemic syndrome caused by interaction of Hb Questembert (alpha1 codon 131, TCT>CCT, Ser>Pro) with an alpha-thalassemia-2 deletion: implications for diagnosis and management.

Abnormal globin chain biosynthesis may result in deficient quantity (thalassemia) or structural variation (abnormal hemoglobins) and traditionally, they represent two phenotypically distinct groups of disorders. However, the phenotypic expression of unstable hemoglobin variants often combine features of thalassemia together with variable peripheral hemolysis. To achieve definitive diagnosis in a child presenting with hemolytic anemia along with features associated with thalassemia intermedia, we evaluated clinical, hematological, biochemical, globin biosynthetic and molecular data. Definitive diagnosis was achieved by DNA analysis which characterized the proband to be a compound heterozygote for a common alpha-thalassemia-2 deletion (3.7 kb) and Hb Questembert (alpha131[H14] Ser>Pro) caused by a C>T mutation in codon 131 of the alpha1 globin gene in trans. The phenotype of thalassemia intermedia with marked dyserythropoiesis, found in patients inheriting alpha-thalassemia mutations along with unstable alpha-globin variants (i.e., alpha-thalassemic hemoglobinopathies), represents a distinct type of thalassemic syndrome. The proband in this study additionally had variable peripheral hemolysis, presumably related to characteristics of the unstable Hb Questembert. There is minimal experience for the management of such atypical cases and this case illustrates that it is probably insufficient to monitor clinical status in patients with such hemoglobinopathies based only on the levels of hemoglobin.

Congenital erythrocytosis with increased erythropoietin level.

The term "absolute erythrocytosis" denotes a heterogeneous group of disorders characterized by an increased red blood cell mass. The authors describe a 20-month-old girl with absolute erythrocytosis. Erythropoietin levels were found to be extremely increased, although extensive evaluation failed to reveal a cause for such an inappropriate increase. Of interest is also the documentation of spontaneous erythroid colony formation in the patient's bone marrow cultures. Although such a finding is considered typical of polycythemia vera, the diagnostic criteria of this myeloproliferative disorder were not met.