ADAM, Zdeněk, Roman HÁJEK, Marta KREJČÍ, Vladimír MAISNAR, Lucie ŘÍHOVÁ, Sabina ŠEVČÍKOVÁ, David BELADA, Michal KAŠČÁK and Marek TRNĚNÝ. Diagnostika a léčba Waldenströmovy makroglobulinemie (Diagnosis and treatment of Waldenström's macroglobulinemia). Transfuze a hematologie dnes. Praha: Česká lékařská společnost J.E.Purkyně, 2014, vol. 20, Listopad 2014/Suppl., p. 7-22. ISSN 1213-5763.
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Basic information
Original name Diagnostika a léčba Waldenströmovy makroglobulinemie
Name (in English) Diagnosis and treatment of Waldenström's macroglobulinemia
Authors ADAM, Zdeněk (203 Czech Republic, guarantor, belonging to the institution), Roman HÁJEK (203 Czech Republic), Marta KREJČÍ (203 Czech Republic, belonging to the institution), Vladimír MAISNAR (203 Czech Republic), Lucie ŘÍHOVÁ (203 Czech Republic), Sabina ŠEVČÍKOVÁ (203 Czech Republic, belonging to the institution), David BELADA (203 Czech Republic), Michal KAŠČÁK (203 Czech Republic) and Marek TRNĚNÝ (203 Czech Republic).
Edition Transfuze a hematologie dnes, Praha, Česká lékařská společnost J.E.Purkyně, 2014, 1213-5763.
Other information
Original language Czech
Type of outcome Article in a journal
Field of Study 30200 3.2 Clinical medicine
Country of publisher Czech Republic
Confidentiality degree is not subject to a state or trade secret
RIV identification code RIV/00216224:14110/14:00080156
Organization unit Faculty of Medicine
Keywords in English bendamustin; bortezomib; rituximab; hyperviscosity; cryoglobulinemia; cold agglutinin disease; monoclonal imunoglobulin related disorders; Waldenström's macroglobulinaemia
Tags EL OK
Changed by Changed by: Ing. Mgr. Věra Pospíšilíková, učo 9005. Changed: 2/1/2015 17:24.
Abstract
Waldenströmova makroglobulinemie (WM) patří do skupiny B-lymfoproliferativních onemocnění. Jedná se o krevní nádorové onemocnění, pro něž je typická lymfoplazmocytární infiltrace kostní dřeně nebo lymfaticke tkáně a přítomnost monoklonálního imunoglobulinu (MIC) typu IgM v séru. Jedná se zatím o nevyléčitelné onemocnění. Rozhodnutí o léčebném přístupu je komplexní. Nemocné je nutné stratifikovat dle rizika a tomu uzpůsobit zvolený léčebný postup.
Abstract (in English)
Therapy of Waldenström's macroglobulinaemia (WM) is indicated in patients with clinically relevant symptoms. Therapeutic plasmapheresis should be performed in cases with hyperviscosity. The intensity of chemotherapy should be adjusted to the degree of cytopenia. Monotherapy with rituximab is recommended in cases with severe cytopenia, also combination of rituximab with dexamethasone should be possible. Patients with symptomatic WM without severe cytopenia should received a rituximab-containing regimens, optimal variant is combination of rituximab + dexamethasone + alkylation drug (such as cyclophosphamide or bendamustine). Possible treatment combinations are for instance R-CHOP (rituximab, cyclophosphamide, vincristine, and prednisone), R-COP (rituximab, cyclophosphamide, and prednisone), or RCD (rituximab, cyclophosphamide, and dexamethasone). The choice of regimen in individual patients will take into consideration performance status, clinical features including renal function, comorbidities and potential candidacy for stem cell transplantation. Chlorambucil and rituximab is possible treatment options for older patients. The choice of treatment of WM relapse depends on the time of treatment response. Retreatment with primary therapy may be appropriate in patients with duration of treatment response at least 2 years. Other treatment possibilities for WM relapse are regimens containing fludarabine, cladribine or bortezomib. Autologous transplantation of peripheral blood stem cells is feasible therapeutic option for relapsed WM in younger, fitter patients with aggressive chemosensitive disease.
Links
NT13190, research and development projectName: Molekulární charakteristika centrozomálních abnormalit a jejich prognostický význam pro pacienty s mnohočetným myelomem
Investor: Ministry of Health of the CR
NT14310, research and development projectName: Buněčné stárnutí u hematoonkologických onemocnění
Investor: Ministry of Health of the CR
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