Qhov sib txawv tseem ceeb ntawm HLH thiab MAS yog tias HLH (hemophagocytic lymphohistiocytosis) yog ib qho mob hnyav, hematologic hematologic mob hnyav, thaum MAS (macrophage activation syndrome) yog ib qho mob hnyav, mob rheumatic.
Cytokine cua daj cua dub tseem hu ua hypercytokinemia. Nws yog ib qho physiological cov tshuaj tiv thaiv nyob rau hauv tib neeg thiab lwm yam tsiaj nyob rau hauv lub innate lub cev tiv thaiv kab mob ua rau ib tug uncontrolled thiab tshaj tawm ntawm cytokines. Cytokines yog pro-inflammatory signaling molecules. Txawm li cas los xij, tam sim ntawd tso tawm ntawm cytokines hauv ntau ntau tuaj yeem ua rau ntau lub cev tsis ua haujlwm. HLH thiab MAS yog ob yam mob uas tuaj yeem ua rau muaj cua daj cua dub cytokine.
HLH yog dab tsi (Hemophagocytic Lymphohistiocytosis) ?
HLH (hemophagocytic lymphohistiocytosis) yog ib qho mob hematologic tsis tshua muaj tshwm sim hauv cov menyuam yaus ntau dua li cov neeg laus. Nws yog ib yam kab mob uas ua rau tuag taus. Nws ua rau mob hnyav heev los ntawm kev loj hlob ntawm cov lymphocytes thiab macrophages. Nquag, HLH yog tus cwj pwm los ntawm kev loj hlob ntawm morphologically benign lymphocytes thiab macrophages, uas tso tawm ntau ntau ntawm cov kab mob cytokines. Yog li, HLH raug cais raws li cytokine storm syndrome.
Figure 01: HLH
Tseem muaj kev xeeb tub thiab tsis tau txais txiaj ntsig ua rau hemophagocytic lymphohistiocytosis. Ntxiv mus, muaj ob hom HLH: thawj thiab theem nrab. Primary HLH yog tshwm sim los ntawm inactivating mutations nyob rau hauv cov noob uas code rau cov proteins uas cytotoxic T hlwb thiab NK hlwb siv los tua cov hom kab mob. Cov noob hloov pauv muaj xws li UNC13D, STX11, RAB27A, LYST, PRF11, SH2DIA, BIRC4, ITK, CD27, thiab MAGT1. Qhov thib ob HLH yog txuam nrog cov kab mob malignant thiab non-malignant uas ua rau tsis muaj peev xwm ntawm lub cev tiv thaiv kab mob los tua cov kab mob EBV. Cov kab mob malignant muaj xws li T cell lymphoma, B cell lymphoma, mob lymphocytic leukemia, thiab lwm yam. Cov kab mob uas tsis yog malignant, ntawm qhov tod tes, muaj xws li cov kab mob autoimmune xws li juvenile idiopathic arthritis, juvenile Kawasaki disease, systemic lupus erythematosus, etc.
HLH cov tsos mob muaj xws li ua npaws, ua rau lub siab loj thiab tus po, cov qog nqaij hlav loj, daj daj ntawm daim tawv nqaij thiab qhov muag, thiab pob khaus. Cov mob no tuaj yeem kuaj tau nrog kev kuaj lub cev, kuaj ntshav, thiab kuaj molecular rau kev hloov pauv noob. Cov kev kho mob rau tus kab mob no suav nrog corticosteroids, tshuaj tiv thaiv kab mob (cyclosporine, methotrexate), tshuaj kho cov tshuaj (etoposide, vincristine), intravenous immunoglobulin, cytokine tsom txoj kev kho, anti-IFN gamma monoclonal antibody, (emapalumab), thiab hloov kho cov tshuaj recombinant. antagonist (Anakinra).
MAS (Macrophage Activation Syndrome) yog dab tsi?
Macrophage activation syndrome (MAS) yog ib qho mob hnyav, ua rau muaj kev phom sij rau lub neej. Nws yog ib tug mob rheumatic mob ntawm me nyuam yaus. Nws tshwm sim feem ntau nrog cov kab mob ntawm cov menyuam yaus idiopathic mob caj dab. Nws kuj tseem cuam tshuam nrog cov kab mob lupus erythematosus, kab mob Kawasaki, thiab cov neeg laus-pib tseem cov kab mob. MAS kuj xav tias zoo ib yam li lwm yam hemophagocytic lymphohistiocytosis. Qhov ua rau tus kab mob no feem ntau yog kis kab mob los yog siv tshuaj.
Figure 02: MAS
Cov tsos mob ntawm tus kab mob no suav nrog ua npaws tsis tu ncua, nkees nkees thiab tsis muaj zog, mob taub hau, tsis meej pem, cov qog nqaij hlav loj, lub siab loj thiab tus po, cov teeb meem los ntshav thiab txhaws, hloov ntshav siab, thiab ntshav qis. Kev kuaj mob ntawm MAS yog los ntawm kev kuaj ntshav, pob txha pob txha aspirate, thiab kev kuaj pom (X-ray, ultrasound, CT-SCAN, MRI). Cov kev xaiv kho mob feem ntau suav nrog cov tshuaj steroids (glucocorticoids), cov tshuaj tiv thaiv kab mob hauv lub cev, cyclosporine (cov tshuaj tiv thaiv kab mob), biologic therapy (Anakinra), thiab tshuaj tiv thaiv kab mob.
Qhov Zoo sib xws ntawm HLH thiab MAS yog dab tsi?
- HLH thiab MAS yog ob yam mob uas tuaj yeem ua rau cua daj cua dub cytokine.
- Ob kab mob feem ntau cuam tshuam rau menyuam yaus.
- Secondary HLH pathophysiologically is similar to MAS.
- Ob kab mob cuam tshuam nrog cov menyuam yaus idiopathic mob caj dab, menyuam yaus Kawasaki kab mob, thiab kab mob lupus erythematosus,
- Lawv muaj kev kho mob zoo sib xws.
Qhov txawv ntawm HLH thiab MAS yog dab tsi?
HLH yog tus kab mob hnyav, ua rau muaj kev phom sij rau lub neej thaum MAS yog tus kab mob rheumatic hnyav, ua rau muaj kev phom sij rau lub neej. Yog li, qhov no yog qhov sib txawv tseem ceeb ntawm HLH thiab MAS. Tsis tas li ntawd, muaj cov ua tau los thiab tsis tau txais txiaj ntsig rau HLH, tab sis tsuas muaj qhov tsis tau txais txiaj ntsig rau MAS.
Cov hauv qab no qhia txog qhov sib txawv ntawm HLH thiab MAS hauv daim ntawv teev npe rau ib sab ntawm kev sib piv.
Summary – HLH vs MAS
HLH thiab MAS yog ob yam mob uas tuaj yeem ua rau muaj cua daj cua dub hauv lub cev. Cov menyuam yaus feem ntau cuam tshuam los ntawm ob qho xwm txheej. HLH yog ib qho mob hnyav, ua rau muaj kev phom sij rau lub neej, thaum MAS yog tus kab mob rheumatic mob hnyav. Yog li, qhov no qhia txog qhov txawv ntawm HLH thiab MAS.
