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Dtsch Med Wochenschr 2017; 142(02): 111-114
DOI: 10.1055/s-0042-121365
Kasuistik
© Georg Thieme Verlag KG Stuttgart · New York

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 Therapy
Anna Hecht
1   Universitätsmedizin Mannheim, III. Medizinische Klinik, Hämatologie und internistische Onkologie, Mannheim
,
Joerg Mezger
2   St. Vincentius Kliniken, Abteilung für Hämatologie, Onkologie, Immunologie und Palliativmedizin, Karlsruhe
,
Martina Gnadler
2   St. Vincentius Kliniken, Abteilung für Hämatologie, Onkologie, Immunologie und Palliativmedizin, Karlsruhe
,
Eva Lengfelder
1   Universitätsmedizin Mannheim, III. Medizinische Klinik, Hämatologie und internistische Onkologie, Mannheim
› Author Affiliations
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Publication History

Publication Date:
23 January 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.

Schlüsselwörter

akute Promyelozytenleukämie (APL) - Differenzierungssyndrom - All-trans Retinsäure (ATRA) - Arsentrioxid (ATO)

Key words

acute promyelocytic leukemia - differentiation syndrome - all-trans retinoic acid (ATRA) - arsenic trioxide (ATO)
 

  • Literatur

  • 1 Sanz MA. Lo Coco F. Martin G. et al. Definition of relapse risk and role of nonanthracycline drugs for consolidation in patients with acute promyelocytic leukemia: a joint study of the PETHEMA and GIMEMA cooperative groups. Blood 2000; 96: 1247-1253
    PubMedGoogle Scholar
  • 2 Lo-Coco F. Avvisati G. Vignetti M. et al. Retinoic acid and arsenic trioxide for acute promyelocytic leukemia. N Engl J Med 2013; 369: 111-121
    CrossrefPubMedGoogle Scholar
  • 3 Burnett AK. Russell NH. Hills RK. et al. Arsenic trioxide and all-trans retinoic acid treatment for acute promyelocytic leukaemia in all risk groups (AML17): results of a randomised, controlled, phase 3 trial. Lancet Oncol 2015; 16: 1295-1305
    CrossrefPubMedGoogle Scholar
  • 4 Frankel SR. Eardley A. Lauwers G. et al. The "retinoic acid syndrome" in acute promyelocytic leukemia. Ann Intern Med 1992; 117: 292-296
    CrossrefPubMedGoogle Scholar
  • 5 Soignet SL. Frankel SR. Douer D. et al. United States multicenter study of arsenic trioxide in relapsed acute promyelocytic leukemia. J Clin Oncol 2001; 19: 3852-3860
    CrossrefPubMedGoogle Scholar
  • 6 Montesinos P. Bergua JM. Vellenga E. et al. Differentiation syndrome in patients with acute promyelocytic leukemia treated with all-trans retinoic acid and anthracycline chemotherapy: characteristics, outcome, and prognostic factors. Blood 2009; 113: 775-783
    CrossrefPubMedGoogle Scholar
  • 7 Sanz MA. Montesinos P. How we prevent and treat differentiation syndrome in patients with acute promyelocytic leukemia. Blood 2014; 123: 2777-2782
    CrossrefPubMedGoogle Scholar
  • 8 De Botton S. Dombret H. Sanz M. et al. Incidence, clinical features, and outcome of all trans-retinoic acid syndrome in 413 cases of newly diagnosed acute promyelocytic leukemia. The European APL Group. Blood 1998; 92: 2712-2718
    PubMedGoogle Scholar
  • 9 Burnett AK. Grimwade D. Solomon E. et al. Presenting white blood cell count and kinetics of molecular remission predict prognosis in acute promyelocytic leukemia treated with all-trans retinoic acid: result of the Randomized MRC Trial. Blood 1999; 93: 4131-4143
    CrossrefPubMedGoogle Scholar
  • 10 Fenaux P. Chastang C. Chevret S. et al. A randomized comparison of all transretinoic acid (ATRA) followed by chemotherapy and ATRA plus chemotherapy and the role of maintenance therapy in newly diagnosed acute promyelocytic leukemia. The European APL Group. Blood 1999; 94: 1192-1200
    CrossrefPubMedGoogle Scholar
  • 11 Tallman MS. Andersen JW. Schiffer CA. et al. All-trans retinoic acid in acute promyelocytic leukemia: long-term outcome and prognostic factor analysis from the North American Intergroup protocol. Blood 2002; 100: 4298-4302
    CrossrefPubMedGoogle Scholar