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Olika typer av Mastocytos

Mastocytos är egentligen ett samlingsnamn för en rad olika sjukdomstillstånd som alla har det gemensamt att patienten har förhöjd halt av Mastceller någonstans i kroppen. 

Generellt kan man säga att barn oftast får den Kutana Mastocytosen, medan vuxna får den Systemiska varianten. Alla de andra typerna som nämns i förteckningen ovan är ytterligt ovanliga. Läs gärna mer om de olika underavdelningarna av Mastocyto genom att klicka på dem ovan.

De allra flesta barn och även de flesta vuxna får relativt beskedliga varianter av av sjukdomen, även om klåda kan vara ett plågsamt gissel för vuxen-patienterna. Men de riktigt allvarliga – ibland rentav livshotande – symptomen drabbar väldigt få. 

Man delar traditionellt in Mastocytos i två huvudtyper:

Ibland talas det också om en tredje huvudtyp:

  • Mastocytom – fasta tumörer.

 

Världshälsoorganisationen WHO gör dessutom följande indelning i undervarianter och bryter ner även dessa ytterligare en nivå i under-klassificeringar:

1.       Cutaneous Mastocytosis (CM) – på svenska Kutan Mastocytos
     
a.       Urticaria Pigmentosa (UP) = Maculopapular Cutaneous Mastocytosis
     
b.       Diffuse Cutaneous Mastocytosis (DCM) – på svenska Diffus Kutan Mastocytos
     
c.       Mastocytoma of Skin – på svenska solitärt hudmastocytom

2.       Indolent Systemic Mastocytosis (ISM) – på svenska Systemisk Mastocytos

3.       SM with Associated Clonal Hematologic Non-Mast Cell-Disease (SM-AHNMD)
     
a.       In SM-AHNMD, both the SM component and the AHNMD component of the disease have to be subclassified. The SM may be ASM or ISM depending on clinical presentations. The AHNMD often is a myeloid leukemia or a myelodysplastic syndrome (MDS).

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