ISSN:
1432-1076
Keywords:
Ivemark's syndrome
;
Asplenia
;
Cyanotic cardiopathy
;
Morphology of erythrocytes
;
Heinz bodies
;
Howell-Jolly bodies
;
Target cells
;
Traumatic hemolysis
;
Fragmentosis
;
Nephrosiderosis
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Description / Table of Contents:
Zusammenfassung Es werden am Beispiel von 4 Fällen mit Ivemark-Syndrom die auf eine Alienie hinweisenden cytologischen Befunde des peripheren roten Blutbildes demonstriert. Diese stellen für die Diagnose der Alienie bzw. des Ivemark-Syndromes ein Leitsymptom dar. Ist die Prognose für Neugeborene und Säuglinge mit Ivemark-Syndrom generell infaust, so wird sie im Einzelfalle dennoch durch eine intensive Betreuung (Schutz vor Infektionen) und evtl. durch palliative kardiochirurgische Eingriffe zu verbessern sein. Dieses setzt die rechtzeitige Diagnose voraus, die bei Beachtung der für eine Alienie charakteristischen Blutbildbefunde und der klinisch-physikalischen Untersuchungsergebnisse auf einfachste Weise, wenigstens als Verdachtsdiagnose, zu stellen ist. Als Besonderheit wird eine in einem unserer Fälle (Fall IV) nachgewiesene Fragmentocytose der Erythrocyten im peripheren Blutbild mitgeteilt, die mit einer Hyperbilirubinämie und einer histologische gesicherten Nierensiderose kombiniert war. Die Fragmentocytose war Ausdruck einer traumatischen Hämolyse bei kompliziertem Vitium cordis congenitum.
Notes:
Abstract In 4 cases of Ivemark's syndrome (one 5-day-old and one 8-day-old female infant, one 43-day-old and one 4-month-old male infant) we looked for the following cytological features of the peripheral red blood which indicate the absence of the spleen: 1. Heinz bodies (denatured hemoglobin) in more than 10‰ of the peripheral erythrocytes (Fig. 1). 2. Howell-Jolly bodies (DNS or nuclear debris) in 1–5‰ or more of the erythrocytes in nonanemic, in 5‰ or more of the erythrocytes in anemic patients (Figs. 2 and 3). 3. A larger than normal number of normoblasts (more than 10 erythrocytes per 100 leukocytes). 4. Siderocytes (more than 3‰). 5. Target cells (Fig. 3). 6. Intraerythrocytic vacuoles (Fig. 4). These abnormalities are basic hematological symptoms of Ivemark's syndrome, but they can be found in disorders other than congenital absence of the spleen: An increased spontaneous production of Heinz bodies can be caused by premature birth, a lack of glucose-6-phosphat-dehydrogenase, or on a hemoglobin anomaly. The production of Heinz bodies is also provoked by drugs or toxins. Target cells are found in hemoglobin anomalies, iron deficiency, and hyperbilirubinemia. The diagnosis of a congenital absence of the spleen or of Ivemark's syndrome can be made, at least tentatively, on the basis of the above findings together with the clinical-physical and X-ray findings (malposition and anomalies in the segmentation of the abdominal and thoracic organs), especially when a stronger than normal fragmentocytosis indicates a complicated congenital heart defect. The heart defect is a major symptom of Ivemark's syndrome and it appeared in all of our cases as a primitive malformation of the heart. The fragmentocytosis (22‰fragmentocytes at the age of 25 days!) found in 1 case (case IV) was combined with an indirect hyperbilirubinemia and a histologically substantiated nephrosisderosis. It was related to a cardiogenic traumatic hemolysis. The prognosis for infants with Ivemark's syndrome is in general poor. However, in individual cases it can be improved through intensive care (protection against infections) and sometimes through palliative cardiologic (surgical) treatment. This requires an early diagnosis, which can be facilitated by careful evaluation of the peripheral red blood picture.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00440030
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