بيانات النشر: Uppsala universitet, Hematologi
Heidelberg Univ, Univ Hosp Mannheim, Dept Hematol & Oncol, Mannheim, Germany.
Univ Hosp RWTH Aachen, Dept Oncol Hematol Hemostaseol & Stem Cell Transpl, Aachen, Germany.;Aachen Bonn Cologne Dusseldorf, Ctr Integrated Oncol, Aachen, Germany.
Univ Groningen, Dept Allergol, Groningen, Netherlands.
Univ Groningen, Univ Med Ctr Groningen, Dept Hematol, Groningen, Netherlands.
Med Univ Gdansk, Dept Allergol, Gdansk, Poland.
Med Univ Gdansk, Dept Dermatol Venereol & Allergol, Gdansk, Poland.
Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.
Med Univ Vienna, Dept Internal Med 1, Div Hematol & Hemostaseol, Vienna, Austria.;Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria.;Univ Vet Med, Univ Clin Small Anim, Dept Univ Clin Compan Anim & Horses, Internal Med Small Anim, Vienna, Austria.
Johannes Kepler Univ Linz, Univ Clin Hematol & Oncol, Linz, Austria.
Johannes Kepler Univ Linz, Kepler Univ Hosp, Allergy Ctr, Dept Dermatol & Venereol, Linz, Austria.
Verona Univ Hosp, Dept Med, Sect Hematol, Verona, Italy.
Verona Univ Hosp, Allergy Unit, Verona, Italy.
Univ Freiburg, Fac Med, Med Ctr Univ Freiburg, Dept Med 1, Freiburg, Germany.;German Canc Consortium DKTK, Partner Site Freiburg, Heidelberg, Germany.;German Canc Res Ctr, Heidelberg, Germany.
Univ Freiburg, Fac Med, Med Ctr Univ Freiburg, Dept Med 1, Freiburg, Germany.;German Canc Consortium DKTK, Partner Site Freiburg, Heidelberg, Germany.;German Canc Res Ctr, Heidelberg, Germany.;Univ Schleswig Holstein, Med Ctr, Dept Hematol & Oncol, Campus Lubeck, Lubeck, Germany.
Univ Freiburg, Fac Med, Med Ctr, Dept Dermatol, Freiburg, Germany.
Stanford Univ, Stanford Canc Inst, Sch Med, Stanford, CA USA.
Fdn IRCCS Policlin San Matteo, Hematol Unit, Pavia, Italy.
Univ Salerno, Div Allergy & Clin Immunol, Salerno, Italy.
Semmelweis Univ, Dept Hematol, Budapest, Hungary.
Univ Padua, Dept Med, Pediat Dermatol Unit, Padua, Italy.
Tech Univ Munich, Sch Med, Dept Dermatol & Allergy, Munich, Germany.
Katholieke Univ Leuven, Dept Microbiol Immunol & Transplantat, Allergy & Clin Immunol Res Grp, Leuven, Belgium.;Univ Hosp Leuven, MASTeL, Leuven, Belgium.
Istanbul Univ, Istanbul Med Sch, Div Hematol, Istanbul, Turkiye.
Univ Hosp Brno, Dept Internal Med Hematol & Oncol, Brno, Czech Republic.;Masaryk Univ, Fac Med, Brno, Czech Republic.
Univ Antwerp, Fac Med & Hlth Sci, Dept Immunol Allergol Rheumatol, Antwerp, Belgium.;Antwerp Univ Hosp, Antwerp, Belgium.
Univ Hosp Graz, Dept Dermatol & Venereol, Graz, Austria.
Univ Basel, Dept Dermatol, Div Allergy, Basel, Switzerland.;Univ Hosp Basel, Dept Biomed, Basel, Switzerland.;Univ Basel, Basel, Switzerland.
Univ Paris 05, Hosp Necker, Assistance Publ Hop Paris, Imagine Inst,French Reference Ctr Mastocytosis, Paris, France.
Univ Paris Sorbonne, Pitie Salpetriere Hosp, Assistance Publ Hop Paris, French Reference Ctr Mastocytosis, Paris, France.
نبذة مختصرة : Certain laboratory abnormalities correlate with subvariants of systemic mastocytosis (SM) and are often prognostically relevant. To assess the diagnostic and prognostic value of individual serum chemistry parameters in SM, 2607 patients enrolled within the European Competence Network on Mastocytosis and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells were analyzed. For screening and diagnosis of SM, tryptase was identified as the most speci fic serum parameter. For differentiation between indolent and advanced SM (AdvSM), the following serum parameters were most relevant: tryptase, alkaline phosphatase, beta 2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein (P < .001). With regard to subvariants of AdvSM, an elevated LDH of ≥ 260 U/L was associated with multilineage expansion (leukocytosis, r = 0.37, P < .001; monocytosis, r = 0.26, P < .001) and the presence of an associated myeloid neoplasm (P < .001), whereas tryptase levels were highest in mast cell leukemia (MCL) vs non-MCL (308 μg/L vs 146 μg/L, P = .003). Based on multivariable analysis, the hazard-risk weighted assignment of 1 point to LDH (hazard ratio [HR], 2.1; 95% confidence interval [CI], 1.1-4.0; P = .018) and 1.5 points each to β2-microglobulin (HR, 2.7; 95% CI, 1.4-5.4; P = .004) and albumin (HR, 3.3; 95% CI, 1.7-6.5; P = .001) delineated a highly predictive 3-tier risk classification system (0 points, 8.1 years vs 1 point, 2.5 years; ≥1.5 points, 1.7 years; P < .001). Moreover, serum chemistry parameters enabled further stratification of patients classified as having an International Prognostic Scoring System for Mastocytosis-AdvSM1/2 risk score (P = .027). In conclusion, serum chemistry pro filing is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.
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