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Schwere therapierefraktäre Osteoporose

Eine seltene Differenzialdiagnose

Severe therapy refractive osteoporosis

A rare differential diagnosis

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Zusammenfassung

Unter einer Mastozytose versteht man eine klonale Vermehrung von Mastzellen. Wir berichten über 4 Patienten mit schwerer Osteoporose bei histologisch gesicherter Mastozytose. Bei den Patienten kam es unter antiresorptiver Therapie zu weiteren Frakturen. Es bestanden starke Knochenschmerzen. Bei therapierefraktären oder ungeklärten Osteoporoseformen ist die systemische Mastozytose eine wichtige Differenzialdiagnose. Eine Urticaria pigmentosa ist nur selten vorhanden, meist handelt es sich um einen isolierten Befall des Knochenmarks. Die Bestimmung der Tryptase im Serum kann Hinweise auf eine systemische Mastozytose geben, beweisend ist nur die Knochenstanze. Die Patienten sind stark frakturgefährdet, die Therapie sollte in spezialisierten osteologischen Schwerpunktzentren DVO erfolgen. Die besten Daten existieren zum Bisphosphonat Zoledronat.

Abstract

Systemic mastocytosis is defined as a clonal increase of mast cells. We report on four patients with severe osteoporosis and histologically confirmed systemic mastocytosis. In spite of antiresorptive therapy the patients developed further vertebral fractures and suffered from ostealgia. Systemic mastocytosis is an important differential diagnosis in patients with therapy refractive and unexplained osteoporosis. Skin involvement (urticaria pigmentosa) is a rare occurrence and in most cases an isolated involvement of bone marrow is present. Determination of serum tryptase can provide indications for systemic mastocytosis but the diagnosis is only confirmed by bone marrow biopsy. There is a high risk of vertebral fractures and patients should be treated in specialized centers. Zoledronic acid can be a therapeutic option for indolent osteoporosis associated with systemic mastocytosis.

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Authors and Affiliations

  1. Fachklinik für Rheumatologie, Orthopädie und Osteologie, Klinik Der FÜRSTENHOF, Osteologisches Schwerpunkt- und Forschungszentrum DVO, Am Hylligen Born 7, 31812, Bad Pyrmont, Deutschland

    M. Gehlen,  A. D. Lazarescu, C. Hinz, N. Schmidt & M. Pfeifer

  2. Campus Medizin, Evangelisches Krankenhaus, Universität Oldenburg, Oldenburg, Deutschland

    M. Schwarz-Eywill

  3. Nordwestdeutsches Rheumazentrum, St.-Josef-Stift Sendenhorst, Sendenhorst, Deutschland

    A. Maier

  4. Helios Klinikum Emil von Behring, Berlin, Deutschland

    M. Werner

  5. Rheumatologische Facharztpraxis Joachimstraße, Hannover, Deutschland

    H. F. Weidemann

Authors
  1. M. Gehlen
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  2. A. D. Lazarescu
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  3. C. Hinz
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  4. N. Schmidt
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  5. M. Pfeifer
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  6. M. Werner
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  7. H. F. Weidemann
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  8. M. Schwarz-Eywill
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  9. A. Maier
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Corresponding authors

Correspondence to M. Gehlen or A. D. Lazarescu.

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M. Gehlen, A.D. Lazarescu, C. Hinz, N. Schmidt, M. Pfeifer, M. Werner, H.F. Weidemann, M. Schwarz-Eywill und A. Maier geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben.

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E. Reinhold-Keller, Hamburg

F. Moosig, Neumünster

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Gehlen, M., Lazarescu, A.D., Hinz, C. et al. Schwere therapierefraktäre Osteoporose. Z Rheumatol 75, 729–735 (2016). https://doi.org/10.1007/s00393-016-0096-0

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  • DOI: https://doi.org/10.1007/s00393-016-0096-0

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