I taksa i itch reng em? A sen a ni em? He natna hmuh tur awm lo (Systemic Mastocytosis) chungchang hi i zir chiang ang u

I taksa pum puiah pawh a chang chuan i itchy thin em? I hmai leh i kawr chu a sen nghal vek a, i hot flush ang maiin i hria em? I pum na, luak chhuak, leh thinlung chak tak i nei em? Heng thilte hi allergy mai maiah i ngai a nih pawhin, a chang chuan he thil hnung lamah hian dinhmun danglam tak a awm thei a, chu chu kan la hre tam lo hle. Vawiin hian chutiang natna hmuh tur awm lo pakhat, Systemic Mastocytosis chungchang kan sawi dawn a ni. Lungngai suh, hei hi awlsam takin i hrethiam ang u.

Systemic mastocytosis tih hi eng nge ni?

A awlsam zawngin sawi ila, hei hi kan taksaa ‘mast cells’ an tih cell chi khat, a pangngai lo taka a punna avanga lo awm natna a ni. Mast cell hi thisen var chi khat a ni. Heng mast cell te hi kan immune system-a sipai angin han ngaihtuah teh. An hna ber chu natna laka min humhim a , kan hliam huna kan dam theihna tura min puih hi a ni . Mahse he natnaah hian heng mast cells te hi kan vun, ruhro , ruh, leh intestines te ang chi taksa peng hrang hrangah a tam lutuk leh a pangngai lo takin an pung khawm thin. Chuti a nih chuan eng nge thleng? Trigger engemaw zat avang hian heng mast cell tam lutuk te hi an thawk nghal a, taksaah ‘histamine’ ang chi chemical tam tak an chhuah tir thin. Chutih lai chuan a hmaa ka sawi tawh ang chi, flushing, itching, leh eczema , te chu a lo lang ta a ni.

Eng thilin nge he natna hi a thlen?

Mi tam tak chuan hei hi thlahtu natna a ni em tih an ngaihtuah thin. Mahse hei hi a tlangpuiin thlahtu natna a ni lo. A tam zawkah chuan kan mast cell-a awm KIT an tih gene-ah a thleng thin.Random change (mutation) avanga lo awm a ni. He gene hian cell thanna control tu protein siam a pui thin. Chuvangin hei hi a inthlak chuan mast cells te chu thunun theih lohvin an lo thang lian tan thin. Heng chhinchhiahnate hi kum eng pawha lang thei mah se, kum upa lam, a bik takin kum 40 leh 50 inkarah a langsar ber a ni .

Symptoms tizualtu triggers

He natna vei zawng zawng hian symptom inang an nei vek lo. Tin, symptoms tichhuaktu trigger te pawh mi hrang hrangah a inang lo thei bawk. Mahse, trigger tlangpui thenkhat chu a hnuaia table-ah hian tarlan a ni. Hengte hi fimkhur a pawimawh hle. Tin, a chang chuan chhan bik awm lovin symptoms a lo lang thei tih hriat reng a pawimawh bawk.

He natna chi hrang hrang leh a lan chhuah dan berte chu engte nge ni?

Systemic Mastocytosis hi chi hrang hrang panga a awm a. I doctor chuan a test ang che a, eng type nge i neih tih a hrilh ang che.

• Indolent Systemic Mastocytosis (ISM): Hei hi a tam ber leh a na lo zawk chi a ni. Natna hi a zual zel a, taksa peng hrang hrang a tichhe nasa lo.
• Smoldering Systemic Mastocytosis (SSM): ISM aiin a na deuh deuh. Symptoms hi a na zawk a, hun kal zelah taksa peng hrang hrang a nghawng thei bawk.
• Systemic Mastocytosis with an Associated Hematological Neoplasm (SM-AHN): Hetiang a nih chuan mastocytosis hi thisen nena inzawm natna dang (e.g. leukemia ) nen a inzawm a ni.
• Aggressive Systemic Mastocytosis (ASM): He chi hi a kal chak hle a, taksa peng hrang hrang, thin, ruh leh ruh te a tichhe thei a ni.
• Mast Cell Leukemia (MCL): Hei hi a tlem ber leh a na ber chi a ni. Ruhro leh thisenah hian mast cell tam tak a awm a.

I natna lan chhuah dan chu i natna chi hrang hrang leh i taksaa mast cell pangngai lo awmna hmun a innghat a ni. Heng a lan chhuah dan tlangpui thenkhat chu hetiang hi a ni:

• Itching, rash , a hring chhuak
• Pum na, pum na, luak chhuak
• Nausea emaw, luak chhuak emaw
• Lu na leh hah lutuk
• Thinlung thawk chak tak
• Thisen sang hniam, luak chhuak, hriatna hloh
• Liver, spleen leh lymph node te a hring chhuak
• Anemia leh thisen chhuahna dinhmun
• Ruh chak lo (Osteoporosis) .
• Rilru lamah chuan ngaihtuahna seng harsa, lungkham fo, emaw, lungngaihna ang chi dinhmun.

Engtin nge natna hi kan hriat theih ang? (Diagnosis) tih a ni.

I doctor chuan i symptoms te a zawt hmasa ang che a, physical exam a ti ang che. Tichuan, a natna hriat chian nan test engemaw zat neih an rawt thei ang:

• Skin biopsy: Vun te tak te chu a natna hmun atanga lak chhuah a ni a, microscope hmangin mast cell tam tak a awm em tih enfiah a ni.
• Bone Marrow Biopsy: Hematologist chuan needle te tak te hmangin i ruhro sample te tak te a la a, a enfiah thin.
• Thisen leh zun test: 1.1.Thisen chhiar kimchang (CBC) hmangin natna dang, thisen tlakchhamna (anemia) te pawh enfiah theih a ni bawk.
• Genetic Test: Thisen emaw tissue sample emaw chu a hmaa kan sawi tawh KIT gene-ah hian danglamna a awm em tih enfiah a ni.
• Imaging test: X-ray, ultrasound, leh CT scan te hian ruh chak lohna leh liver leh spleen ang chi taksa peng hrang hrang a hring chhuak hriat theih nan a pui thei a ni.
• Endoscopy emaw Colonoscopy: Tube te tak te, camera inbun a ni a, chu chu pum chhung emaw, intestines emaw enfiah nan, ulcer ang chi thil dik lo awm leh awm loh enfiah nan hman a ni.

Enkawlnate chu engte nge ni?

Doctor chuan i natna lan chhuah dan te, taksa peng hrang hrang a nghawng dan te, natna chi hrang hrang a zirin enkawl dan tur a ruat ang.

• Antihistamines: Heng damdawi te hian vun na, sen, leh pum na te ang chi thil te a control thei a ni.
• Epinephrine: Allergy na tak (anaphylaxis) a awm chuan hormone hman thin a ni.
• Proton Pump Inhibitors: Pum acid control a, pum na ang chi thil a tihziaawm.
• Mast Cell Stabilizers: Mast cell atanga histamine chhuak tur titawp thei damdawi.
• Steroids: Vun leh thawkna lama natna lan chhuahnaah a pui thei.
• Targeted Therapy: Natna kaihhnawih gene emaw protein emaw bik target tu damdawi. Hengte hi natna lanchhuahna na tak nei tan chuan a ngai mai thei.
• Chemotherapy: He enkawlna hi natna darh chak tak leh taksa peng hrang hrang tihchhiatna nasa tak thlentuah chuan a tul thei a ni.
• Stem Cell Transplant: Enkawlna tur ngaihtuah thin, a tlem hle.

Systemic Mastocytosis, mast cells, vun rawng, anaphylaxis, anaphylaxis, KIT gene, histamine te a awm bawk