Hairy Cell Leukemia (HCL) nthuav tawm Nrog Kev Kho Mob Sib Nqus: Cov Ntaub Ntawv Qhia Thiab Kev Tshawb Xyuas Cov Ntaub Ntawv Ntawm Qhov Tsis tshua muaj Rheumatological Qhia Txog Tus Kab Mob Hematological

Abstract

Cov plaub hau cell leukemia (HCL) yog ib qho mob tsis tshua muaj tshwm sim uas feem ntau cuam tshuam rau pob txha pob txha, cov ntshav peripheral, thiab tus po. Feem ntau nthuav tawm cov yam ntxwv ntawm HCL yog splenomegaly lossis cytopenias ua rau qaug zog, kis kab mob, lossis hemorrhagic tshwm sim. Cov tsos mob ntawm cov nqaij mos los yog pob txha tsis tshua muaj. HCL tsis tshua muaj tshwm sim nrog lub cev tiv thaiv kab mob polyarthritis. Qhov kev nthuav qhia no tuaj yeem tsis meej pem rau lwm cov koom haum pathological, xws li Felty's syndrome, thiab tuaj yeem sib txawv ntawm qhov no nrog cov pob txha pob txha. Cov ntaub ntawv tshaj tawm no saib mus rau hauv qhov tsis tshua muaj kev nthuav qhia ntawm HCL nyob rau hauv uas transient polyarthritis ntawm lub hauv caug yog cov tsos mob tshwm sim.

Cistanche tuaj yeem ua raws li kev tiv thaiv kev qaug zog thiab lub zog ua kom muaj zog, thiab cov kev tshawb fawb sim tau pom tias decoction ntawm Cistanche tubulosa tuaj yeem tiv thaiv daim siab hepatocytes thiab endothelial hlwb puas hauv cov nas ua luam dej hnyav, txhawb kev qhia ntawm NOS3, thiab txhawb cov kab mob siab glycogen. synthesis, yog li exerting los tiv thaiv qaug zog. Phenylethanoid glycoside-nplua nuj Cistanche tubulosa extract tuaj yeem txo cov ntshav creatine kinase, lactate dehydrogenase, thiab lactate qib, thiab nce qib hemoglobin (HB) thiab piam thaj hauv ICR nas, thiab qhov no tuaj yeem ua lub luag haujlwm tiv thaiv kev qaug zog los ntawm kev txo qis cov leeg nqaij. thiab ncua lub lactic acid enrichment rau lub zog cia hauv nas. Compound Cistanche Tubulosa ntsiav tshuaj ua rau lub sijhawm ua luam dej hnyav, nce siab glycogen cia, thiab txo qis qib urea tom qab kev tawm dag zog hauv cov nas, qhia nws cov nyhuv tiv thaiv kev qaug zog. Lub decoction ntawm Cistanchis tuaj yeem txhim kho kev ua siab ntev thiab ua kom lub cev qaug zog hauv kev tawm dag zog nas, thiab tuaj yeem txo qhov siab ntawm cov ntshav creatine kinase tom qab kev tawm dag zog thiab ua kom lub cev nqaij daim tawv nqaij ntawm cov nas ib txwm muaj tom qab kev tawm dag zog, uas qhia tau hais tias nws muaj cov teebmeem. ntawm kev txhim kho lub cev muaj zog thiab tiv thaiv qaug zog. Cistanchis kuj tseem ua rau lub sijhawm muaj sia nyob ntawm cov nas uas muaj nitrite-poisoned thiab txhim kho lub siab ntev tiv thaiv hypoxia thiab qaug zog.

Nyem rau ntawm Tiredness

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Pawg: Internal Medicine, Oncology, Hematology

Ntsiab lus: rheumatoid mob caj dab, luv v600e, kab mob mimics, felty's syndrome, neutropenia, splenomegaly, mob caj dab, pancytopenia, sib koom o, plaub hau cell leukemia

Taw qhia

Cov plaub hau cell leukemia (HCL) yog qhov tsis tshua muaj neeg paub tab lymphoid B-cell malignancy uas cuam tshuam rau cov ntshav peripheral, spleen, thiab pob txha pob txha [1]. Nrog rau lub hnub nyoog nruab nrab ntawm kev kuaj mob ntawm 55 thiab txiv neej predominance, nws yog kwv yees li 2% ntawm tag nrho cov leukemias thiab tsawg dua 1% ntawm tag nrho cov lymphoid neoplasms [2,3]. HCL classically nthuav tawm nrog splenomegaly thiab pancytopenia [4]. Splenomegaly yog ib qho classic ntawm HCL thiab palpable spleen feem ntau yog ib qho kev tshawb nrhiav ntawm lub cev [5].

Peb nthuav tawm ib rooj plaub ntawm HCL pom tom qab tus neeg mob tau nthuav tawm qhov mob hnyav ob sab hauv lub hauv caug thiab o. Muab lub xub ntiag ntawm polyarthritis, splenomegaly, thiab neutropenia, qhov kev nthuav qhia no tuaj yeem yooj yim tsis meej pem nrog Felty's syndrome, ib qho kev tshwm sim ntawm rheumatoid mob caj dab (RA). Kev kuaj HCL xav tau cov pob txha pob txha biopsy thiab aspirate, nrog rau kev tiv thaiv kab mob los ntawm kev ntws cytometry [2,6]. Kev txheeb xyuas ntawm "cov plaub hau" cov hlwb hauv cov pob txha pob txha smear thiab cov qhab nia immunologic raws li CD qhia yuav tsum tau txiav txim siab txog kev kuaj mob HCL [1]. HCL variant (HCL-V), ib qho kab mob sib txawv, yog qhov sib txawv ntawm HCL. Qhov kev hloov pauv no tuaj yeem pom los ntawm qhov sib txawv hauv kev tiv thaiv kab mob thiab txheeb xyuas qhov tsis muaj BRAF kev hloov pauv thiab monocytopenia [4]. HCL-V yog txuam nrog cov kab mob hauv pob txha infiltrative uas tuaj yeem tshwm sim raws li kev sib koom ua ke; Qhov tshwm sim no tau txiav txim tawm hauv peb rooj plaub [7].

Case Presentation

Ib tug txiv neej muaj hnub nyoog 55-xyoo-laus Caucasian nrog keeb kwm kev kho mob yav dhau los ntawm kev tswj tsis tau ntshav siab thiab rog rog tau nthuav tawm rau chav kho mob xwm txheej ceev (ER) nrog mob, liab liab, thiab o ntawm ob sab hauv caug, nrog rau qaug zog. Nws cov tsos mob ntawm lub hauv caug yog insidious txij li thaum pib, pib li ntawm peb hnub ua ntej kev nthuav qhia thiab kev loj hlob sai. Nws thawj zaug tsim mob hnyav thiab o ntawm nws lub hauv caug sab xis, ua raws li cov tsos mob zoo sib xws hauv lub hauv caug sab laug hnub tom qab. Nws tau piav qhia qhov mob ntawm lub hauv caug hnyav li 10/10 thiab hais tias nws taug kev tsis tau vim qhov mob, uas ua rau nws tuaj rau hauv ER. Nws kuj tau tshaj tawm tias muaj tus mob ua npaws nrog ua daus no txog ib hnub dhau los. Nws tsis lees paub qhov poob phaus lossis raug mob tsis ntev los no.

Cov cim tseem ceeb ntawm kev nthuav qhia yog qhov kub ntawm 97.8 degree F, ntshav siab ntawm 134/83 mmHg, mem tes ntawm 101 beats / min, thiab ua pa ntawm 18 cycles / min. Kev kuaj lub cev thaum lub sij hawm nkag tau zoo kawg nkaus rau ob sab hauv lub hauv caug o, erythema, rhiab, thiab me me ob sab pedal edema.

Nws cov ntshav tshawb nrhiav pom cov qe ntshav dawb suav ntawm 1,860/µL (3,{3}},5{18}}/µL) nrog rau qhov tseeb neutrophil suav ntawm 130/µL (1,{ {7}}, 000/µL), hemoglobin ntawm 8.6 g/dL (13.5-17.5 g/dL), hematocrit ntawm 26.8% ({16}}%), thiab platelet suav ntawm 43,00 /µL (150,{20}},000/µL) (Table 1). Peb tau txais tus neeg mob rau kev ntsuam xyuas thiab kev tswj xyuas ntxiv.

Thaum nkag mus, vim muaj qhov tsis txaus ntseeg ntawm sepsis hauv qhov chaw ntawm neutropenic fever, tus neeg mob tau muab tso rau hauv kev sib cais nrog kev ceev faj neutropenic thiab tau pib ntawm empiric IV tshuaj tua kab mob (cefepime). Cov ntshav pib ua haujlwm kuj tau nthuav tawm D-dimer ntawm 3522 ng / mL, uas ua rau CT angiogram (CTA) ntawm lub hauv siab thiab venous Doppler ultrasound ntawm ob txhais ceg. Thaum CTA pab txiav txim rau pulmonary embolism, nws tshwm sim tau txheeb xyuas splenomegaly ntawm 17.8 cm (Daim duab 1) thiab sub-centimeter mediastinal thiab hilar lymph nodes (Daim duab 2).

CT ntawm lub plab thiab pelvis corroborated lub splenomegaly (Daim duab 3) thiab qhia hepatomegaly thiab sub-centimeter sab laug periaortic thiab superior mesenteric lymph nodes. CT-guided pob txha hlwb biopsy tau ua tiav thiab cov qauv tau raug xa mus rau immunohistochemistry, ntws cytometry, thiab kev kawm cytogenetic. Tus neeg mob tau sau tseg tias muaj hemoglobin ntawm 6.8 g / dL nyob rau hnub thib ob ntawm kev lees paub thiab tau txais cov ntshav hloov nrog ib chav ntawm cov qe ntshav liab. Lactic acid, procalcitonin, thiab ob kab lis kev cai ntawm cov ntshav tsis zoo. Tus neeg mob lub hauv caug mob thiab o tuaj yeem txhim kho tau zoo thiab yuav luag daws tau tag nrho nyob rau hauv thawj peb hnub ntawm kev nkag. Yog li ntawd, kev ua haujlwm rheumatological ntxiv tsis tau ua raws. Sib nrug los ntawm kev qaug zog me ntsis, tus neeg mob tseem asymptomatic thiab afebrile thoob plaws hauv tsev kho mob.

Cov pob txha pob txha smear (Daim duab 4) tau nthuav tawm qee cov qog ntshav qog ntshav uas tshwm sim los ntawm HCL hlwb thiab, yog li ntawd, raws li kev kuaj mob ntawm HCL. CD 20 thiab BRAF V600E stains tau zoo. Flow cytometry txoj kev tshawb fawb txhawb nqa lub tswv yim ntawm HCL nrog 29% lambda monotypic B hlwb zoo rau CD11c, CD19, CD20, CD25, thiab CD103. Thaum kawg, peb tso tus neeg mob tawm nrog kev saib xyuas sab nraud hematology-oncology.

Kev sib tham

Classic HCL yog ib qho kab mob lymphoproliferative mob ntev uas tshwm sim los ntawm kev nkag mus rau malignant B hlwb nrog cov plaub hau zoo li qhov projections ua rau cov pob txha pob txha tsis ua haujlwm [1]. HCL paub tias yog tus cwj pwm los ntawm kev muaj kab mob, splenomegaly, lossis cytopenia thaum lub sijhawm kuaj mob. Hauv 2011, nrog kev siv tag nrho-exome sequencing (WES), BRAF V600E somatic mutation tau txheeb xyuas hauv tus neeg mob nrog HCL. Qhov kev hloov pauv no tau raug txheeb xyuas tom qab txog li 70-100% ntawm HCL mob [2]. Kev kuaj mob feem ntau tau lees paub nrog tartrate-resistant acid phosphatase staining los ntawm cytochemistry los yog los ntawm cov pob txha pob txha biopsy qhia cov plaub hau classic [5]. Cov cim cytometry uas zoo hauv HCL suav nrog CD11c, CD25, CD103, thiab CD123, ntxiv rau cov cim B-cell, CD19, CD20, lossis CD22 [4]. HCL-V suav txog li 10% ntawm HCL mob [2]. HCL-V tsis qhia txog CD25 thiab CD200, thiab cov immunophenotype yog cov laus B-cell nrog zoo rau CD103 thiab CD11c B-cell antigens [2,7]. Thaum thawj zaug tau piav qhia hauv xyoo 1958, HCL tau cuam tshuam nrog kev muaj sia nyob tsis txaus ntseeg. Los ntawm xyoo 1990, nrog rau kev siv cov tshuaj purine analogs xws li cladribine, HCL tau hloov mus ua ib qho kev kho mob zoo tshaj plaws hauv keeb kwm mob qog noj ntshav [1].

Cov tsos mob ntawm HCL muaj xws li plab tsis xis nyob los yog tag nrho (uas tshwm sim los ntawm splenomegaly loj), qaug zog, tsis muaj zog, kis kab mob, thiab los ntshav tshwm sim [5]. Qhov tsis muaj peev xwm kom tau txais cov pob txha pob txha nrog kev xav, piv txwv li, "qhuav kais", feem ntau qhia nrog HCL [4]. Ob peb tus neeg mob yuav asymptomatic ntawm kev nthuav qhia [5]. Ib tug granulocytes suav nyob rau hauv 500/µL yog txuam nrog kev loj hlob ntawm lub neej hem tus kab mob nyob rau hauv li ib nrab ntawm cov neeg mob kuaj HCL [5].

Tsis tshua muaj HCL kuj nthuav tawm hauv daim ntawv ntawm lymphomas hauv cov pob txha thiab cov ntaub so ntswg [8]. Kev tshuaj xyuas rov qab ntawm 37 qhov xwm txheej los ntawm Westbrook thiab Golde tau tshaj tawm tias rau tus neeg mob muaj cov tsos mob sib koom [9]. Riambourg et al. tau tshaj tawm tias ntawm 27 qhov xwm txheej ntawm HCL, lawv tau tswj hwm, ib qho nthuav tawm nrog migrating arthralgia raws li cov tsos mob pib [10]. Qhov kev nthuav qhia no tau raug ntaus nqi los ntawm hematological malignancy nws tus kheej los yog lub cev tsis muaj zog [10]. Qhov kev sib txawv ntawm qhov kev tshwm sim no feem ntau tau ua rau kev kuaj mob tsis meej thiab muaj peev xwm ua tsis tau zoo ntawm qhov kev mob no [8,10]. Tsis tas li ntawd, ntau cov ntaub ntawv xov xwm tau piav qhia txog kev nthuav qhia RA nrog HCL. Nyob rau hauv cov ntaub ntawv ntawm lub cev tiv thaiv kab mob inflammatory mob caj dab, cov tsos mob ntawm mob caj dab yuav tshwm sim ua ntej los yog tom qab pib ntawm classic leukemic tsos mob [11]. Cov ntaub ntawv qhia los ntawm L'Hirondel et al. piav qhia txog cov tsos mob polyarthritis luv luv daws tau sai nrog alpha{11}} interferon therapy [12]. Tsim kom muaj kev kuaj mob feem ntau nyuaj raws li HCL kuj tau pom tias ua rau tsis muaj kab mob polyarthritis nrog synovial kua txheeb xyuas cov plaub hau hlwb raws li pom los ntawm Zervas li al. [13]. HCL-V tau raug tshaj tawm tias muaj kab mob sib kis mob vim yog leukemic infiltration ua rau pob txha expansion [7].

Felty's syndrome, ib qho kev tshwm sim ntawm RA cuam tshuam nrog neutropenia thiab splenomegaly, feem ntau yog tshwm sim los ntawm T-cell lymphoproliferative disorders [10]. Qhov no classic triad ntawm cov tsos mob ua rau nws yog thawj qhov kev kuaj mob sib txawv hauv peb rooj plaub. Interestingly, HCL tau lees paub tias yog qhov tsis tshua muaj tshwm sim rau RA [10]. Zervas et al. piav qhia txog rooj plaub ntawm HCL tom qab tsim RA [13]. Taylor et al. tshaj tawm ib rooj plaub ntawm seropositive inflammatory mob caj dab uas tsim nyob rau hauv ib tug neeg mob HCL uas tau txais kev kho alpha interferon [14]. Muaj cov lus ceeb toom ntawm Felty's syndrome tau nyuaj los ntawm kev txhim kho HCL [15]. Facchini et al. kuj tau tshaj tawm tias HCL tuaj yeem ua rau lub sijhawm ntev ntawm RA [16].

Peb tus neeg mob tau nthuav tawm nrog ob sab o thiab qhov mob uas ua rau muaj kev sib tw hnyav heev ntawm kev kuaj lub cev. Cov tsos mob hnyav txaus ua rau nws txwv tsis pub thauj mus los. Tus neeg mob BMI ntawm 30.1 kg / m2 ua rau muaj kev sib tw ntxiv rau palpate ib qho splenomegaly ntawm kev kuaj lub cev. Pancytopenia ntawm kev ua haujlwm ntawm cov ntshav thiab qhov teeb meem nrhiav pom ntawm splenomegaly ntawm CTA ua rau muaj kev tsis txaus siab ntawm hematological malignancy thiab coj mus rau qhov kev kuaj mob tom ntej ntawm HCL. Flow cytometry zoo rau CD25 ntxiv rau qhov zoo BRAF V600E stain tshwm sim txiav tawm HCL-V. Qhov kev daws teeb meem ntawm qhov o nrog kev saib xyuas kev ntsuas qhia tau hais tias cov tsos mob yog tej zaum ib qho kev tiv thaiv kev tiv thaiv ib ntus es tsis yog qhov tshwm sim ncaj qha ntawm malignancy xws li leukemic infiltration.

Cov lus xaus

Cov ntaub ntawv no hais txog qhov tseem ceeb ntawm kev lees paub qhov tsis txaus ntseeg ntawm rheumatologic manifestations ntawm cov kab mob hematologic, tshwj xeeb tshaj yog qhia txog qhov tsis tshua muaj tshwm sim ntawm kev sib koom tes o raws li thawj cov tsos mob ntawm HCL. Kev nthuav qhia ntawm HCL nrog cov tsos mob tsis tshwj xeeb xws li qaug zog thiab sib koom ua ke tuaj yeem ua rau muaj kev tsis txaus ntseeg yog tias tsis tau tshawb xyuas meej. Yog li, cov kws kho mob yuav tsum xav txog qhov kev kuaj mob dav dav thaum ntsuas cov neeg mob uas muaj cov tsos mob ntawm kev mob caj dab hauv qhov teeb tsa ntawm qhov tsis tau piav qhia cytopenia.

Cov ntaub ntawv ntxiv

Qhia tawm

Tib neeg cov ntsiab lus: Kev pom zoo tau txais lossis zam los ntawm txhua tus neeg koom hauv txoj kev tshawb no. Kev tsis sib haum xeeb ntawm kev txaus siab: Ua raws li daim ntawv qhia txog ICMJE, txhua tus kws sau ntawv tshaj tawm cov hauv qab no: Cov ntaub ntawv them nyiaj / kev pabcuam: Txhua tus kws sau ntawv tau tshaj tawm tias tsis muaj kev txhawb nqa nyiaj txiag los ntawm ib lub koom haum rau kev xa ua haujlwm. Kev sib raug zoo nyiaj txiag: Txhua tus kws sau ntawv tau tshaj tawm tias lawv tsis muaj kev sib raug zoo nyiaj txiag tam sim no lossis hauv peb lub xyoos dhau los nrog rau txhua lub koom haum uas yuav muaj kev txaus siab rau cov haujlwm xa tuaj. Lwm yam kev sib raug zoo: Txhua tus kws sau ntawv tau tshaj tawm tias tsis muaj lwm yam kev sib raug zoo lossis kev ua ub no uas tuaj yeem tshwm sim los cuam tshuam rau kev xa tawm.

Cov ntaub ntawv

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