Waldenström macroglobulinemia

Waldenström macroglobulinemia (WM) ke mofetše oa li-lymphocyte tsa B (mofuta oa sele e tšoeu ea mali). WM e amahanngoa le tlhahiso e fetelletseng ea liprotheine tse bitsoang lithibela-mafu tsa IgM.

WM e bakoa ke boemo bo bitsoang lymphoplasmacytic lymphoma. Ena ke mofetše oa lisele tse tšoeu tsa mali, moo lisele tsa 'mele tsa B li qalang ho arohana kapele. Lebaka la 'nete la tlhahiso e ngata haholo ea lesole la mmele la IgM ha le tsejoe. Hepatitis C e ka eketsa kotsi ea WM. Hangata liphetoho tsa lefutso li fumanoa liseleng tse kotsi tsa B.

Ho hlahisa li-antibodies tse ngata tsa IgM ho ka baka mefuta e fapaneng ea mathata:

• Hyperviscosity, e etsang hore mali a be motenya haholo. Sena se ka thatafalletsa mali ho phalla methapong e menyenyane ea mali.
• Neuropathy, kapa tšenyo ea methapo, ha lesole la 'mele la IgM le sebetsana le lisele tsa methapo.
• Phokolo ea mali, ha lesole la 'mele la IgM le tlama lisele tse khubelu tsa mali.
• Lefu la liphio, ha lesole la 'mele la IgM le kenya lisele tsa liphio.
• Cryoglobulinemia le vasculitis (ho ruruha ha methapo ea mali) ha lesole la 'mele la IgM le theha libaka tsa boits'ireletso ba mmele ka ho pepesehela serame.

WM e fumaneha seoelo haholo. Batho ba bangata ba nang le boemo bona ba lilemo tse fetang 65.

Matšoao a WM a ka kenyelletsa tse latelang ho tse latelang:

• Ho tsoa mali a marenene le a nosebleeds
• Pono e fifetseng kapa e fokotsehileng
• Letlalo le leputsoa menoaneng kamora ho pepesehela serame
• Ho tsekela kapa pherekano
• Ho longoa habonolo ha letlalo
• Mokhathala
• Letšollo
• Ho ba lerootho, ho hlohlona, ​​kapa bohloko bo tukang matsohong, maotong, menoaneng, menoaneng, litsebeng kapa linkong
• Moferefere
• Litšoelesa tse ruruhileng
• Ho theola boima ba 'mele ka boomo
• Ho lahleheloa ke pono ka leihlo le le leng

Tlhahlobo ea 'mele e ka senola spleen e ruruhileng, sebete le li-lymph node. Tlhahlobo ea mahlo e ka bonts'a methapo e atolositsoeng ho retina kapa ho tsoa mali ho tsoa ho retina (hemorrhages).

CBC e bonts'a palo e tlase ea lisele tse khubelu tsa mali le liplatelete. Khemistri ea mali e ka bontša bopaki ba lefu la liphio.

Teko e bitsoang serum protein electrophoresis e bonts'a boemo bo phahameng ba lesole la 'mele la IgM. Methati hangata e phahame ho feta limiligrama tse 300 ka desilitara (mg / dL), kapa 3000 mg / L. Ho tla etsoa tlhahlobo ea immunofixation ho bontša hore lesole la mmele la IgM le tsoa mofuteng o le mong oa sele, (clonal).

Tlhahlobo ea viscosity ea serum e ka tseba hore na mali a se a le motenya. Hangata matšoao a hlaha ha mali a le motenya ka makhetlo a mane ho feta a tloaelehileng.

Biopsy ea moko oa masapo e tla bonts'a palo e eketsehileng ea lisele tse sa tloaelehang tse shebahalang joalo ka li-lymphocyte le lisele tsa plasma.

Liteko tse ling tse ka etsoang li kenyelletsa:

• Protheine ea moroto oa lihora tse 24
• Kakaretso ea protheine
• Ho itšireletsa mafung ka morong
• T (thymus e fumanoeng) palo ea lymphocyte
• Masapo x-ray

Batho ba bang ba nang le WM ba ekelitseng lithibela-mafu tsa IgM ha ba na matšoao. Boemo bona bo tsejoa e le smoldering WM. Ha ho hlokahale kalafo ntle le ho latela ka hloko.

Ho batho ba nang le matšoao, kalafo e ikemiselitse ho fokotsa matšoao le kotsi ea ho ba le tšenyo ea setho. Ha ho na kalafo e tloaelehileng ea hajoale. Mofani oa hao oa tlhokomelo ea bophelo a ka u khothaletsa hore u kenye letsoho tekong ea bongaka.

Plasmapheresis e tlosa lithibela-mafu tsa IgM maling. E boetse e laola kapele matšoao a bakoang ke ho tenya ha mali.

Meriana e ka kenyelletsa corticosteroids, motsoako oa meriana ea chemotherapy le anti-monoclonal antibody ho lisele tsa B, rituximab.

Autologous stem cell transplant e ka buelloa ho batho ba bang ba nang le bophelo bo botle ka tsela e ngoe.

Batho ba nang le palo e tlase ea lisele tse khubelu kapa tse tšoeu tsa mali kapa liplatelete ba ka hloka litšelo kapa lithibela-mafu.

Karolelano ea bophelo ke lilemo tse ka bang 5. Batho ba bang ba phela lilemo tse fetang 10.

Ho batho ba bang, lefu lena le ka hlahisa matšoao a fokolang mme la tsoela pele butle.

Mathata a WM a ka kenyelletsa:

• Liphetoho ts'ebetsong ea kelello, mohlomong li lebisa ho coma
• Ho hloleha ha pelo
• Ho tsoa mali ka mpeng kapa malabsorption
• Mathata a pono
• Hives

Ikopanye le mofani oa hau haeba matšoao a WM a hlaha.

Waldenström macroglobulinemia; Macroglobulinemia - e ka sehloohong; Lymphoplasmacytic lymphoma; Monoclonal macroglobulinemia

• Mosebetsi
• Masole a mmele

Kapoor P, Ansell SM, Fonseca R, le al. Ho hlahlojoa le ho tsamaisoa ha Waldenström macroglobulinemia: Mayo stratification ea macroglobulinemia le tataiso ea likotsi (mSMART) tataiso ea 2016. JAMA Oncol. 2017; 3 (9): 1257-1265. PMID: 28056114 e phatlalalitsoeng.ncbi.nlm.nih.gov/28056114/.

Setsi sa Rajkumar SV. Mathata a sele ea mali. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 178.

Treon SP, Castillo JJ, Hunter ZR, Merlini G. Waldenström macroglobulinemia / lymphoplasmacytic lymphoma. Ka: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Melao-motheo le Tloaelo ea Motheo. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 87.