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DOI: 10.1055/s-0042-121365
Sehr schweres Differenzierungssyndrom bei Niedrigrisiko akuter Promyelozytenleukämie – eine Tücke der differenzierenden Therapie
Very Severe Differentiation Syndrome in Low Risk Acute Promyelocytic Leukemia – A Peril of Differentiating TherapyPublikationsverlauf
Publikationsdatum:
23. Januar 2017 (online)
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Zusammenfassung
Anamnese, Untersuchungen und Diagnose Eine junge Patientin stellte sich wegen spontaner Hämatome im Krankenhaus vor. Aufgrund der Kombination von Panzytopenie und Koagulopathie im Blut sowie den beweisenden zyto- und molekulargenetischen Untersuchungen des Knochenmarks wurde eine Niedrigrisiko akute Promyelozytenleukämie (APL) diagnostiziert.
Therapie und Verlauf Unter der Kombinationstherapie mit ATRA und ATO entwickelte die Patientin ein schweres Differenzierungssyndrom bei Hyperleukozytose. Dies konnte nur durch klinische Expertise und den massiven Ersatz von Blutprodukten und Gerinnungsfaktoren stabilisiert werden.
Folgerung Der Fall verdeutlicht die Schwierigkeiten und Gefahren der APL-Induktionstherapie auch bei günstiger Ausgangslage trotz neuer, insgesamt risikoarmer Therapien.
Abstract
History, examinations and diagnosis A young patient consulted the hospital because of spontaneous hematomas. The combination of pancytopenia and coagulopathy in the blood and the proving cytogenetic and moleculargenetic examinations of the bone marrow lead to the diagnosis of low-risk acute promyelocytic leukemia (APL).
Therapy and clinical course During the combination therapy with ATRA and ATO, the patient developed a severe differentiation syndrome and hyperleukocytosis. Management of the condition was only possible due to clinical expertise and massive substitution of blood products and clotting factors.
Conclusion The case illustrates the difficulty and dangers of APL induction therapy even with a favorable initial clinical presentation despite the generally low toxicity of new therapies.
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