Tumor Lysis Syndrome (TLS) Panyima pa Chemotherapy anyi Steroids: Hyperkalemia, Hyperuricemia, Hypocalcemic Tetany, ne Rasburicase¶

Disama dia Tumor Lysis Syndrome (TLS) i disama dia tshimpitshimpi didi mua kunyanga muoyo mu malu a mashi ne a kansere. Bidi bienzeka 12 too ne ku 72 meba kunyima kua dibanga dia tshimuma tshia cytotoxic, monoclonal anticorps (rituximab, obinutuzumab), targeted BH3-mimetics (venetoclax), CAR-T cell therapy, radiation, anyi mene dose umua wa systemic corticosteroids (prednisone anyi dexametone) udi kabidi mua kuepuka kumpala kua eruption luondapu luonso ludi lutuadija mu masama a mashi adi enda lubilu lukole bu mudi: Burkitt lymphoma, Acute Lymphoblastic Leukemia (ALL), Acute Myeloid Leukemia (AML) ne bungi bua globules blanc, ne Non-Hodgkin Lymphoma wa mushinga mukole. Padi miliyo ya selile ya tumor mibi itshibuka musangu umue, idi iteka bintu bidi munda muayi buludiludi mu mashi, bienza bua tshipepele tshikole tshia metabolisme tshia bitupa binayi: Hyperkalemia mukole (potassium \(> 6.0\text{ mmol/L}\), tshidi tshifikisha ku tshibungubungu tshia T-wave ne tshipupu tshia tshimpitshimpi tshia mu mashi disama dia mutshima), hyperphosphatemia mikole (phosphorus \(> 4.5\text{ mg/dL}\) mu bantu bakole anyi \(> 6.5\text{ mg/dL}\) mu bana), hyperuricemia mikole (uric acid \(> 8.0\text{mg/dL}\)), ne dikosolola dia DNA Hypocalcemie (calcium \(< 7.0\text{ mg/dL}\) as released phosphate udi ulamakaja calcium udi wenda unyunguluka mu calcium phosphate microcrystals idi kayiyi mua kusunguluka idi ikanga tubules ya mifuba pamue ne kristale ya aside wa urique, idi ifila disama dia mifuba dikole dia mu mifuba, tengopas, ne bipupu). Shi mubedi udi mutuadije luondapu lua kansere anyi manga a steroïde, anyi udi ne disama dia kansere ka mu mashi anyi dia lymphome, udi ne disama dia mashi difiike anyi dikese, disama dia mutshima ne disama dia nsukadi, disama dia mifuba anyi disama dia bianza, dikuma dia muoyo, dipanga dia lungenyi, anyi disama dia tshimpitshimpi, ubikile muaba uudi musombele nomba ya tshimpitshimpi diakamue. Tangila kabidi mikanda yetu ya bana betu pa disama dia tshiadi, rhabdomyolyse, ne mashi mafiike, disama dia tshimpitshimpi ne disama dia tshimpitshimpi diambuluisha dia kumpala, ne disama dia vena cava supérieure (SVC) ne disama dia pericardial tamponade dibi.

Tshiudi ne bua kuenza tshia kumpala ntshia kuya ne mubedi ku lupitadi diakamue bua kuenza 12-lead ECG (dikonkonona dia hyperkalemic cardiac toxicity ne hypocalcemic QTc prolongation), kutuadija aggressive potassium-free, calcium-free intravenous isotonic crystalloid crystalloid (250,000 to \(mL/m2/day\)) bua kumbusha aside wa urique ne phosphate wa calcium mu tubules ya mu tshiadi, kufila Rasburicase (urate oxidase, kunyima kua dikonkonona dia dipanga dia G6PD) bua kujikija aside wa urique udiku mu mashi mu mêba 4, ne **Cbey-Phosphate produit Mukenji: KUENA mua kufila calcium gluconate mu mishipa bua disama dia hypocalcémie didi kadiyi ne bimanyinu mu Tumor Lysis Syndrome, bualu kusakidila calcium padi phosphate wa mu mashi muvula kudi kufikisha ku disama dia calciphylaxis ne dikuluka dia mabue adi kaayi mua kupingana munda mua mifuba.

Enza nunku mpindieu¶

1. Bikila nimero wa lupitadi lua muaba unudi anyi ndaku ku tshibambalu tshia lupitadi tshidi pabuipi nenu diakamue pikala mubedi wa disama dia kansere ka mu mashi, dia lymphome anyi dia tumor mukole mu matuku 1 anyi 7 a ntuadijilu wa luondapu (anyi steroïdes) mupeta tshimanyinu tshia TLS. Ela 911 mu États-Unis ne mu Canada, 999 mu Royaume-Uni, mu matunga a ku Mputu onso mu Australie, anyi tangila tshilaminu tshietu tshia buloba bujima tshia nomba ya tshimpitshimpi Shi mubedi udi ne dipuekesha dia mashi (oligurie) anyi mashi adi ne matutu/a nsongo, disama dia mutshima dikole, disama dia tshiadi, anyi dipanga dia bukole, diteketa dia mifuba anyi dikuma dia mutshima, disama dia bianza ne dia makasa (tetany), anyi disama dia tshiadi.
2. Lekela manga onso a potassium ne phosphore, NSAIDs, ne manga adi kaayi ne potassium diakamue. Kulekedi mubedi unua mayi a bimuma adi ne potassium wa bungi (mayi a orange, mayi a koko, mayi a tomate), wangate mafuta a electrolyte adi ne potassium anyi phosphore anyi phosphore udi kayi ne inflamatoire manga (ibuprofen, naproxen), adi apangisha mishipa ya mu tshiadi ne akolesha disama dia tshiadi.
3. Dimuija dia EMS ne dia Hospital Triage bua kupeta STAT 12-Lead ECG mu minite 5 ya difika ne kuzola STAT TLS Lab Panel (Potassium, Phosphorus, Uric Acid, Ionized & Total Calcium, Creatinine, ne LDH). Hyperkalemia i tshintu tshidi tshishipa bantu lukasa mu Tumor Lysis Syndrome; tshikebelu tshia lukasa tshia ECG tshia 12-lead tshidi tshitangila T-waves mile, P-waves mipepele, PR prolongation, anyi sine-wave QRS widening ku divula dia potassium, ne kabidi QTc prolongation ku secondary hypocalcémie.
4. Ikala mushindike ne: baminganga ba mu lupitadi kabatu bafila calcium mu mishipa nansha kakese bua disama dia hypocalcémie didi kadiyi ne bimanyinu padi phosphore mu mashi mikale mivule to. Dimuija bena mu tshisumbu tshia baminganga bikala mubedi ne disama dia Tumor Lysis Syndrome: pa kumbusha padiye ne bimanyinu bia hypocalcémie bidi mua kumushipa (tetany, laryngopasme, sezures, anyi Q-severe arrhythmia), IV calcium gluconate is strictly withheld. Kupuekesha phosphore wa mu mashi kumpala (ku diambuluisha dia IV hydratation, phosphate binders, anyi dialyse) kudi kuambuluisha bua serum calcium ikale muakane nkayende kayi wenza bua mifuba ikale dibue to.
5. Dikonkonona dia malu a kale a dipanga dia G6PD kumpala kua kufila Rasburicase, ne Teka tu-tube tua mashi tua aside wa uric panyima pa Rasburicase diakamue pa mâyi a mashika. Bikala mubedi ne Dipanga dia Glucose-6-Phosphate Dehydrogenase (G6PD) (didi ditamba kuikala kudi babedi ba mu Afrike wa ku Mputu wa ku Est, wa ku Sud-Est, ne wa ku Sud-Est ancestry), Rasburicase is strictly contraindicated bualu dikosolola dia enzyme dia uric acid mu allantoin didi dipatula hydrogène peroxyde (\(\text{H}_2\text{O}_2\)), didi dijula hemolytic anemia ne methemoglobinemia.
6. Ujadike ne: lupitadi kaluena luenza mudimu ne bicarbonate de sodium mu mashi pa tshibidilu to. Nansha muvua mikanda ya kale ilomba bua kuela bicarbonate de sodium mu mashi bua kulama pH wa mu mashi pa mutu pa 7,0 bua kujikija aside wa urique, mikenji ya lelu ya malu a kansere (ASCO, BSH, ESMO) **ikandika dilengeja dia mashi pa tshibidilu bualu T ankaline mu tshisuku pH wa tshisuku tshia alkaline udi pa mutu pa 6.5 udi wenza bua mabwe a calcium phosphate atuluke misangu dikumi lukasa mu tubules ya mu tshiadi ne udi wenza bua calcium udi ne ion apuekele mu mashi (tetany udi unyanguka).

Mutshi wa dipangadika: dimanya dia disama dia Tumor Lysis Syndrome (TLS) ne dijadikulula dia lukasa¶

flowchart TD
    A["Mubedi udi ne disama dia kansere ka mashi, dia lymphome, anyi dia tumor wa bujitu bua bungi (kumpala kua diondapibua anyi mêba 12 too ne ku 72 panyima pa diondapa dia manga, steroïdes, Venetoclax anyi Rituximab)"] --> B{"Enza diakamue disungula dia ku luseke lua bulalu: 12-Lead ECG + Urine Output + STAT TLS Blood Panel (K+, PO4, Uric Acid, Ca2+, Creatinine, LDH)"}
    B -->|2 or more Cairo-Bishop Lab Abnormalities Only (Uric Acid >= 8 mg/dL, K+ >= 6.0 mmol/L, PO4 >= 4.5 mg/dL, Ca2+ <= 7.0 mg/dL)| C["LABORATORIE TUMOR LYSIS SYNDROME (LTLS)"]
    B -->|Lab TLS PLUS >= 1 Clinical Complication: Acute Kidney Injury (Cr >= 1.5x ULN / Oliguria), Cardiac Arrhythmia, OR Seizure / Tetany| D["CLINICAL TUMOR LYSIS SYNDROME (CTLS): Life Threating Medical Emergency"]
    C --> E["Start Aggressive Potassium-Free / Calcium-Free IV Isotonic Crystalloid (0.9% Normal Saline at 200 to 300 mL/hr or 3 L/m2/day to keep Urine Output >= 100 mL/hr [2 mL/kg/hr])"]
    D --> E
    E --> F{"Potassium wa mu mashi udi >= 6.0 mmol/L ANYI T-Waves / Wide QRS udi pa ECG anyi?"}
    F -->|YES: Hyperkalemic Crisis| G["MUSHINDU WA MUOYO WA LUMU: 1. IV Calcium Gluconate 10% (10 to 30 mL) bua kujadika mutshima; 2. IV Regular Insuline (10 units) + Dextrose 50% (25-50 g) + Nebulized Albuterol; 3. Oral Sodium Zirconium Cyclosilicate (Lokelma) anyi Patiromer"]
    F -->|NO: Potassium < 6.0 mmol/L| H{"Tangila tshipiminu tshia aside wa uric, mudimu wa mifuba, ne nsombelu wa G6PD"}
    G --> H
    H -->|Uric Acid >= 8 mg/dL OR Clinical TLS OR High-Risk Tumor (AND No G6PD Deficiency)| I["Ufila IV RASBURICASE (0.2 mg/kg IV mu minite 30; tshitupa tshimue tshia 3 mg too ne ku 6 mg tshidi tshikumbane misangu ya bungi): Udi ukudimuna aside wa uric udiku bua kulua Allantoin udi mua kunyanguka mu mêba 4 (Chill tubes ya mashi ya matuku atshilualua pa ICE)"]
    H -->|Known or Suspected G6PD Deficiency| J["KUPESHI RASBURICASE (Njiwu ya Hemolyse & Methemoglobinemia): Ingidija IV/Oral Allopurinol (anyi Febuxostat) + Aggressive IV Hydration + Early Hemodialysis"]
    I --> K{"Tangila nsombelu wa phosphore ne calcium mu mashi"}
    J --> K
    K -->|Asymptomatic Hypocalcemia (Ca2+ <= 7.0 mg/dL) with High Phosphate| L["KUPESHI IV CALCIUM TO. Kuondopa disama dia hyperphosphatemia ne IV hydratation + oral non-calcium phosphate binders (Sevelamer) bua kukepesha Ca x PO4 produit"]
    K -->|Symptomatic Hypocalcemia (Tetany, Chvostek/Trousseau sign, Laryngospasm, Seizures, or Arrhythmia)| M["Pesha dose mukesa wa gluconate wa calcium mu IV udi ukengela anu bua kuimanyika disama dia tetany/tshipupu + kudilongolola bua hemodialyse wa lukasa / CRRT"]
    L --> N{"Hyperkalemie refractaire, Hyperphosphatémie severe (> 10 mg/dL), Fluid Overload, anyi Oliguric Renal Failance?"}
    M --> N
    N -->|YES| O["KUYIKIJA BUA BUALU BUA BUALU BUA BUALU: Dipanda dia mashi dia lukasa anyi diondapangana dia tshimpitshimpi dia tshiadi (CRRT)"]
    N -->|NO: Urine Output >= 100 mL/hr & Labs Improving| P["Tungunuka ne ICU Telemetry ne upingaje TLS Lab Panel kunyima kua mêba 4 anyi 6"]

Pathophysiology: bua tshinyi selile ya kansere mishipa idi ifila tshipepele tshikole tshia mishindu inayi¶

Ma selile a tshimpumpu mabi adi ne bungi bua bungi bua potassium munda mua selile, phosphore munda mua selile (misangu 4 mipite ya selile idi kayiyi ne makanda a mubidi mimpe), ne aside nucleique purine (ADN ne ARN) bua lubilu lua ditapuluka dia selile. Padi manga a tshimuma, a steroïde, anyi a anticorps misunguluke itshibula membrane ya selile ya miliyare ya selile ya tumor musangu umue, bukole budi nabu mifuba bua kupatula bintu budi bujika mu mêba makese:

THE FOUR-WAY INTRACELLULAR RELEASE IN TUMOR LYSIS SYNDROME
=======================================================================
               [RUPTURED LEUKEMIA / LYMPHOMA CELLS]
                                |
       +------------------------+------------------------+
       |                        |                        |
       v                        v                        v
1. POTASSIUM (K+)       2. PURINE NUCLEIC ACIDS    3. PHOSPHORUS (PO4)
   Floods extracellular    (Adenosine & Guanosine)    (4x higher in tumor
   blood (> 6.0 mmol/L)    Converted by Xanthine      cells; > 4.5 mg/dL)
       |                   Oxidase --> URIC ACID         |
       v                   (> 8 to 15 mg/dL)             v
   Resting cardiac              |                  Binds free blood Ca2+
   membrane potential           |                  when [Ca x PO4 > 60]
   depolarizes                  |                        |
       |                        |                        +------------+
       v                        v                        v            v
   PEAKED T-WAVES,      Uric Acid Crystals       Calcium Phosphate  4. SECONDARY
   WIDE QRS, SINE       precipitate in acidic    Crystals plug         HYPOCALCEMIA
   WAVE & SUDDEN        distal renal tubules     renal tubules &       (< 7.0 mg/dL)
   CARDIAC ARREST                \               microvasculature       |
                                  \                     /               v
                                   v                   v           TETANY, SEIZURES,
                              ACUTE OLIGURIC RENAL FAILURE (AKI)   LARYNGOSPASM &
                              (Stops K+, PO4 & Uric Acid Excretion) LONG QTc
=======================================================================

Bua tshinyi tshintu tshia calcium-phosphate (\(\text{Ca} \times \text{PO}_4 > 60\text{ mg}2/\text{dL}2\)) ntshilumbu tshisokoka mu TLS¶

Mu disama dia Tumor Lysis Syndrome, hypocalcemia ki mmufumina ku dipanga dia calcium mu mubidi mujima to; bidi bifumina ku hyperphosphatemia munene udi wiba calcium wa tshianana mu mashi. - Dîba dionso didi tshipeta tshia makumi tshia Total Serum Calcium (\(\text{mg/dL}\)) tshivudijibue ne Serum Phosphorus (\(\text{mg/dL}\)) tshipita \(60\text{ mg}2/\text{dL}2\) (anyi \(4.8\text{mg/dL}\)2\(} calcium), ne phosphate udi ulua **calcium phosphate (\)\text{Ca}_3(\text{PO}_4)_2$)** udi kayi mua kunyanguka munda mua interstitium wa renal, tubules renal, systeme wa dilombola dia mutshima, ne microvasculature (metastatic calciphylaxis). - Bua tshinyi kufila calcium mu mashi bua muntu udi ne calcium mukese udi kayi ne bimanyinu kudi ne njiwu: Bikala munganga udi ne meji mimpe mumone luapolo lua mu laboratware ludi luleja calcium wa mu mashi wa \(6.4\text{ mg/dL}\) pamue ne phosphore wa mu mashi wa \(10.0\text{ mg/dL\)}\(xt} (xtexte\$}s \text{PO}_4 = 64\)) ne udi wela calcium gluconate mu mushipa padi mubedi kayi ne bimanyinu, calcium udibu bele udi ulamakaja diakamue ku phosphate udi wenda, ne udi ubandisha \(\text{Ca} \times \text{PO}_4\) pa mutu pa 80 ** calcium stones munda mua mifuba, bikanga dipatuka dia masu bionso**.

Caire-Bishop diagnostic criteria: Laboratory TLS vs. Clinical TLS¶

Mikenji ya malu a kansere ya pa buloba bujima (ASCO, ESMO, ne Société britannique pour la haematologie) idi yenza mudimu ne Cairo-Bishop Classification bua kumanya TLS wa mu laboratware ne TLS wa mu lupitadi udi wenzeka pankatshi pa matuku 3 kumpala ne matuku 7 panyima pa dibanga dia luondapu lua diluisha disama dia kansere:

Mmalu kayi a kansere adi ne njiwu mikole ya disama dia Tumor Lysis?¶

1. Malu mabi adi ne njiwu mikole (> 5% ya bungi; Rasburicase ne IV Hydration Prophylaxis ya mu lupitadi idi ne bua kuenzeka):
2. Burkitt Lymphoma / Burkitt Leukemia ne Lymphoblastic Lymphoma.
3. Acute Lymphoblastic Leukemia (ALL) na mashi matoke (WBC) count \(\ge 100,000/\mu\text{L}\) anyi baseline LDH \(\ge 2\times\) ULN.
4. Acute Myeloid Leukemia (AML) ne WBC count \(\ge 100,000/\mu\text{L}\).
5. Diffuse Large B-Cell Lymphoma (DLBCL) ne disama dia bungi (\(\ge 10\text{ cm}\) mass) ne baseline LDH \(\ge 2\times\) ULN.
6. Chronic Lymphocytic Leukemia (CLL) ne bungi bua lymphocyte (\(> 25,000/\mu\text{L}\)) anyi lymphocyte \(\ge 5\text{ cm}\) udi utuadija Venetoclax (BCL-2 inhibitor).
7. Masama a njiwu ya pankatshi (1% too ne ku 5% ya bungi):
8. AML ne WBC \(25,000\text{ to }100,000/\mu\text{L}\); BONSO ne WBC \(< 100,000/\mu\text{L}\); tumor mikole idi ne bukole bua tshimuma (Small Cell Lung Cancer, Metastatic Medulloblastoma, Neuroblastoma, Testicular Germ Cell Tumors); Multiple Myeloma anyi CLL muondapibua ne CAR-T anyi proteasome inhibitors ne dipanga dia mudimu wa tshiadi.

Difuanyikija dia manga: Allopurinol ne Febuxostat ne Rasburicase¶

Lungenyi lua mushinga mukole mu malu a luondapu lua lukasa ne a disama dia kansere ndua kumvua bua tshinyi Allopurinol kena mua kupuekesha aside wa urique udi mumane kuenza mu mashi, kadi Rasburicase udi unyanga aside wa urique udiku mu mêba makese menemene a 4:

PURINE CATABOLISM PATHWAY: ALLOPURINOL vs. RASBURICASE
=======================================================================
  [Tumor Cell DNA/RNA Purines]
               |
               v
        [Hypoxanthine]
               |  <-- XANTHINE OXIDASE (Blocked by ALLOPURINOL / FEBUXOSTAT)
               v      *Stops NEW Uric Acid from forming, but causes Xanthine
          [Xanthine]   to build up (Xanthine is poorly soluble & can still
               |       form Xanthine kidney stones)
               |  <-- XANTHINE OXIDASE (Blocked by ALLOPURINOL / FEBUXOSTAT)
               v
         [URIC ACID]  (Poorly soluble in urine; forms sharp crystals)
               |
               |  <-- RASBURICASE (Recombinant Urate Oxidase Enzyme)
               |      *Directly cleaves EXISTING Uric Acid into Allantoin
               |      + Releases H2O2 (Hydrogen Peroxide byproduct -> Lethal
               v        hemolysis if patient has G6PD DEFICIENCY)
         [ALLANTOIN]  (5 to 10 TIMES MORE SOLUBLE in urine than Uric Acid;
                       flushed effortlessly out of the kidneys in 4 hours)
=======================================================================

Mukenji wa diambuluisha dia lukasa lukasa mu lupitadi bua TLS mujadika¶

1. Difila dia mayi mu mushipa ne tshibumba tshia diurétique tshia tshijengu¶

• Isotonic Crystalloid Volume Expansion: Start potassium-free, calcium-free, phosphate-free isotonic crystalloid, typically 0.9% Normal Saline (anyi 0.45% half-normal saline / D5W with 0.45% NaCl oncemic volume), to 00300 mL/dîba mu bantu bakole (anyi \(2,500\text{ to }3,000\text{ mL/m}2/\text{day}\) mu bana) bua kulama tshipatshila tshikole tshia dipatuka dia mashi dia at least \(100\text{ too ne ku }200\text{ mL/h} mu bantu bakole betu (\)2xt\t mL/kg/dîba}$ mu bana) ne bujitu bua tshisuku \(\le 1.010\).
• Mudimu wa IV Loop Diuretics (Furosemide): Anu panyima pa dijimija dia bungi bua mashi mu mishipa dimane kulongolodibua ne saline, pikala dipatuka dia mashi dipueka muinshi mua \(100\text{ mL/hour}\) anyi bimanyinu bia divula dia mayi bimueneka, ela **IV Furosemide (\(0.5\text {text{\)1}{mL/hour}\(}\)g/kg. bua kulama bungi bua mâyi adi apita mu tubule ne kukolesha dipatuka dia potassium ne calcium. (Kupeshi furosemide nansha kakese kudi mubedi udi kayi ne mâyi mu mubidi anyi udi ne disama dia tshiadi to.)

2. Disungila dia bintu bia nzembu mu tshimpitshimpi bilondeshile malu a kumpala¶

1. Tshintu tshia kumpala: Hyperkalemia udi mua kunyanga muoyo (\(K+ \ge 6.0\text{ mmol/L}\) anyi ECG Changes)
2. Tshitupa A (Kujadika tshitupa tshia mutshima mu minite 1 anyi 3): Pesha **IV Calcium Gluconate 10% (\(10\text{ to }30\text{ mL}\) [\(1\text{ to }3\text{ grams}\)] IV mu minite 5 to 10) (Anyi Calcium IV \(10\text{ mL}\) ku diambuluisha dia mulongo wa pankatshi). Note: Nansha mudi phosphate wa mu mashi muikale ne TLS wa bungi, mashintuluka a ECG a hyperkalemic (T-waves a bungi, QRS mualabale) adi aleja ne: IV calcium udi ne bua kuikalaku bua kuepuka disama dia tshiadi dia lukasa.
3. Tshitupa B (Kupingana ne potassium munda mua selile mu minite 15 to ne 30): Pesha IV Insuline wa pa tshibidilu (\(10\text{ units}\) IV push) kumpala kua IV Dextrose 50% (\(50\text{ mL} = 25\text{\)} glucose (anyi glucose) \(0.1\text{ units/kg}\) insuline + \(0.5\text{ g/kg}\) dextrose mu bana) PLUS High-Dose Nebulized Albuterol / Salbutamol (\(10\text{ to }20\text{ mg}\) continuous nebulization).

4. Tshitupa C (Umbusha potassium mu mubidi): Ela bintu bia potassium bia mu tshifu bidi bienza mudimu lukasa (Sodium Zirconium Cyclosilicate [Lkelma] 10 g ku mishiku misangu isatu ku dituku anyi Patiromer; kuepuka sodium polystyrene sulfonate [**Kayeolit ne sorlabite ne colone bua kufua ne colone njiwu), ufila IV Furosemide pikala euvolemic, ne ufila Emergent Hemodialysis.

5. Tshintu tshia kumpala: Hyperuricemie (Rasburicase Protocol ne "Ice-Bath Tube" Rule)

6. Tshikebelu tshia dipanga dia G6PD (muyuki wa disama, njiwu ya tshisamba, anyi tshikebelu tshia lukasa tshia G6PD; kutangila tshipiminu tshia mashi a ku mpenga bua mibidi ya Heinz/tuselile tua bite ne kutangila bungi bua methemoglobine pikala dipanga dia oxygène ne \(\text{PaO}_2\) dienzeka kunyima kua difila dia manga).
7. Udi mua kufila IV Rasburicase (\(0.2\text{ mg/kg}\) IV mu minite 30, anyi dose umue wa \(3\text{ mg}\) anyi \(6\text{ mg}\) IV kudi bantu bakole).

8. Mukenji wa tshibombi tshia mashi ne mayi a mashika kunyima kua Rasburicase: Padi Rasburicase wenda unyunguluka mu mashi a mubedi, mashi onso adibu bakoka mu tshibombi tshia laboratware tshia luya lua mu nzubu ne atungunuke ne kuikala ne aside wende wa urique mu tshibombi tshia tshikebelu mu njila wa kupatula aside wa urique munda mua tshibombi tshia tshikebelu mu njila wa kupatula aside wa urique anyi zele ** pa tshibidilu tshipeta tshia laboratoire wa aside nansha mudi aside wa mubedi mulelela mu muoyo utshidi anu mubandile. Mashi onso adibu bangate bua kuteta aside wa urique kunyima kua difila dia Rasburicase adi ne tshia kuangata mu tshiamu tshia héparine tshidibu banji kuenza mashika, kutshiteka diakamue mu tshina tshia mâyi a mashika ku luseke lua bulalu, kutshiela mu tshiamu tshia mashika (\(4 degrés Celsius\)), ne kutshikonkonona mu mêba 4.

9. Tshia kumpala 3: Hyperphosphatemia ne hypocalcémie udi uleja bimanyinu

10. Hyperphosphatemia (\(\ge 4.5\text{ mg/dL}\) mu bantu bakole): Kukandika phosphorus yonso mu biakudia ne mu IV, kulama diuresis dia bungi dia mâyi a mu IV, ne kufila non-calcium oral phosphate binders ne biakudia anyi ku diambuluisha dia NG tube, nangananga Sevelate carbonide/carbonide (\(800\text{ to }1,600\text{ mg}\) misangu isatu ku dituku) anyi lanthanum carbonate (kuenza mudimu nansha kakese ne calcium acetate anyi calcium carbonate binders, bidi bivudija \(\text{Ca} \times \text{PO}_4\) product).

11. Symptomatic Hypocalcemia Only: Shi mubedi upeta carpopedal tetany, laryngospasm, seizures, nansha severe QTc prolongation, infuse IV Calcium Gluconate 10% (\(1\text{ to }2\text{ grams}\) [\(10\text{ to {0}txt \text mL}\)] mu bantu bakole, anyi \(50\text{ too ne ku }100\text{ mg/kg}\) mu bana) bitekete bitekete mu minite 10 too ne ku 20 pa dilonda dia ECG ditungunuka, wenza mudimu ne anu dose mukese udi ukengela bua kuimanyika bimanyinu, ki mbua kulongolola nomba wa laboratware to.

12. Tshia kumpala 4: Diondapangana dia kumpala dia tshiadi (Hemodialyse anyi CRRT)

13. Kuindidi uremic coma bua kutuadija dialyse mu TLS to. Bu mudi selile ya tumor itungunuka ne kuela potassium ne phosphate mu mashi lukasa kupita mudi luondapu mua kubijikija padi mashi apatuka, Emergent Intermittent Hemodialysis (udi ujikija potassium ne uric acid lukasa) udi ulonda kudi Continuous Renal Replacement Therapy (CRVRT/HRT, udi ukandika hyperphomie ne hyperkalemia) udi utuadija diakamue bua:
    • Dikola dia bungi (\(K+ > 6.0\text{ to }6.5\text{ mmol/L}\)) nansha mudi insuline/albuterol.
    • Disama dia bungi dia phosphatemie (\(\text{PO}_4 > 10\text{ mg/dL}\) [\(3.2\text{ mmol/L}\)]) anyi disama dia hypocalcemie didi dileja ne tshipeta tshia bungi tshia \(\text{Ca} \times \text{PO}_4\) (\(> 70\text{t mg}2/\text{dL}2\)).
    • Oliguria anyi anuria ne volume wa bungi udi kauyi wandamuna ku diuretiques.

Nomba ya tshimpitshimpi ya mu tshijengu ne mikenji ya bibambalu¶

• États-Unis ne Canada: Ela 911 diakamue bua mubedi yonso wa disama dia kansere udi ne disama dia oliguria, disama dia mutshima, disama dia mifuba, disama dia tshiadi, anyi disama dia tshimpitshimpi panyima pa diondapangana dia manga, steroïdes anyi venetoclax. Lomba bua kuya ku lupitadi ne ICU, Nephrology (24-hour Hemodialysis/CRRT), ne Medical Oncology, bilondeshile mikenji ya American Society of Clinical Oncology (ASCO) ne NCCN Tumor Lysis Syndrome.
• Royaume-Uni: Ela 999 bua disama dia mutshima, dia tshimpitshimpi, anyi dia tshiadi, anyi ubikile ku telefone wa lupitadi luebe 24-hour Acute Oncology Service (AOS) diakamue. Londa mikenji ya British Society for Hematology (BSH) ne NICE bua TLS risk stratification, IV hydration (\(3\text{ L/m}2/\text{day}\) kakuyi potassium), ne difila dia Rasburicase.
• Buobumue bua matunga a ku Mputu: Ela 112 mu matunga onso adi mu matunga a ku Mputu. Bibambalu bia kansere bia ku Mputu bidi bilonda mikenji ya Société européenne pour la oncologie médicale (ESMO) ne Association européenne de hematologie (EHA) bua dikuba ne dilama dia TLS.
• Australie ne Nouvelle-Zélande: Ela 000 mu Australie anyi 111 mu Nouvelle-Zélande bua kuya mu lupitadi lua tshimpitshimpi ludi ne malu a mashi/a kansere ne a dialyse, bilondeshile eviQ (Cancer Institute NSW) mikenji ya luondapu bua Tumor Lysis Syndrome.
• Asie-Pacifique: Ela 119 mu Japon ne mu Corée du Sud, 995 mu Singapour, 999 mu Malaysie ne mu Hong Kong, anyi 112 / 108 mu Inde bua kuya diakamue ku nzubu wa luondapu lua kansere.

Nkonko itubu bela misangu ya bungi¶

Bua tshinyi disama dia Tumor Lysis Syndrome didi dienzeka padi tshimuma tshienza mudimu bimpa?¶

Bualu bua dikema, disama dia Tumor Lysis Syndrome didi dienzeka anu bualu selile ya kansere idi ifua lukasa lukasa bua diondapangana dimpe (anyi bualu tumor udi wenda ukola ne unyanguka lukasa nkayende). Munda mua selile yonso wa kansere ka mashi anyi ka lymphome mudi bintu bia bungi bia potassium, phosphore, ne bintu bidi bienza ADN (purines). Padi luondapu lua tshimuma, manga a steroïde, anyi manga kampanda bu mudi venetoclax anyi rituximab abutula miliyare ya selile ya kansere mu mêba 12 anyi 72, potassium, phosphore ne ADN yonso ya munda idi ibuela mu mashi dîba dimue dimue, ne idi ipita bukole bua mifuba bua kuyipatula.

Ntshinyi tshidi tshilema tshia electrolyte mu disama dia tumor lysis tshidi mua kunyanga muoyo diakamue?¶

Hyperkalemia (potassium wa bungi mu mashi udi ne njiwu mipite pa 6.0 mmol/L) ke njiwu ya lukasa ne ya lufu mu disama dia tumor lysis. Padi miliyo ya selile ya tshimpumpu mitshibuka ipatula potassium wa munda mu mashi eku mifuba mibuikila musangu umue ne aside wa urique ne tshiamu tshia calcium phosphate, bungi bua potassium mu mashi budi mua kuvula mu mêba makese. Bungi bua potassium budi bunyanga mudimu wa nzembu wa mutshima, buenza bua T-waves mile pa ECG ne buenzeja muoyo bua kuimanyika tshimpitshimpi (fibrillation ventriculaire anyi asystole) kakuyi didimuija, anu padibu bamuondopa diakamue ne IV calcium, insuline ne glucose, ne dialyse.

Bua tshinyi bidi ne njiwu bua kufila calcium mu mishipa bua kulongolola tshikebelu tshia mashi tshidi ne calcium mukese mu disama dia Tumor Lysis Syndrome pikala mubedi kayi ne bimanyinu?¶

Mu disama dia Tumor Lysis Syndrome, calcium wa mu mashi a mubedi udi upueka anu bualu bungi bua bungi bua phosphore budi bupatuka mu selile ya kansere mifue budi bulamakaja ne calcium udi mu mashi. Padi tshipeta tshia calcium wa mu mashi tshivudijibua ne phosphore wa mu mashi tshipita 60, calcium ne phosphore bidi bilua mabue makole adi kaayi amueneka bimpe (mabue a calcium phosphate) adi akanga tu-tube tua tshisuku tua mu mifuba. Munganga yeye muele calcium wa pa mutu mu mushipa anu bua kuenza bua bungi bukese bua mu laboratware bumueneke buimpe padi phosphore utshidi anu muulu, calcium udibu bele au udi ulamakaja ne phosphore ne ukudimuka mabue a calcium phosphate munda mua mifuba, ne udi ukebesha dipanga dia mifuba didi kadiyi mua kupingana. IV calcium udi upeshibua bua calcium mukese mu TLS anu pikala mubedi ne bimanyinu bia njiwu bu mudi disama dia mifuba, disama dia tshiadi, disama dia tshiadi, anyi dishintuluka dia rythme wa mutshima.

Ndishilangana kayi didi pankatshi pa Allopurinol ne Rasburicase, ne bua tshinyi badi benza mudimu ne Rasburicase mu njiwu?¶

Allopurinol i tshintu tshia kudikuba natshi tshidi tshipangisha enzyme xanthine oxidase bua mubidi kauyi mua kuenza aside wa urique mupiamupia to, kadi kena ne bukole bua kutshibula aside wa urique udi kale mu mashi to (udi wangata matuku 2 anyi 3 bua bungi buawu kupueka). Rasburicase i tshintu tshidi tshibuela mu mashi (recombinant urate oxidase) tshidi tshiluisha buludiludi aside wa urique udiku mu mashi ne utshikudimuna mu tshintu tshidibu babikila ne: allantoin, tshidi tshinyanguka misangu 5 too ne ku 10 mu mashi kupita aside wa urique. Dose umua wa Rasburicase mu IV udi upuekesha aside wa urique mu mashi pabuipi ne zero mu mêba 4, udi ulama mifuba ku dikanga dia aside wa urique.

Bua tshinyi baminganga badi ne tshia kukontonona dipanga dia G6PD kumpala kua kufila Rasburicase?¶

Padi Rasburicase utshibula aside wa urique mu allantoine mu mashi, tshienzedi tshia tshimfuanyi tshidi tshipatula hydrogène peroxyde (H2O2) bu tshintu tshidi tshipatuka buludiludi. Ma globules rouges adi ne makanda a mubidi adi enza mudimu ne enzyme udibu babikila ne: G6PD (glucose-6-phosphate dehydrogenase) bua kujikija hydrogène peroxyde bua kayinyangi selile to. Ino, kudi babedi badi baledibua ne dipanga dia G6PD (didi ditamba kumueneka kudi bantu ba mu Afrike, ba mu Mediterane, ba ku Moyen-Orient ne ba mu Asie wa ku Sud-Est), globules rouges yabu kayena mua kujikija dibutuka dia peroxyde d’hydrogène to. Kupesha mubedi udi kayi ne G6PD Rasburicase kudi kuenza bua globules rouges yabu ipatuke (disama dikole dia hemolytique) ne kukudimuna hemoglobine wabu mu methemoglobine, udi kayi mua kuambula oxygène.

Bua tshinyi baminganga bakalekela kuenza mudimu ne sodium bicarbonate mu mishipa bua kuenza bua mashi ikale alkaline mu disama dia Tumor Lysis Syndrome?¶

Bidimu bia bungi bishale, baminganga bavua babueja bicarbonate de sodium bua kuenza bua mashi ikale alkaline (pH pa mutu pa 7,0) bualu aside wa urique utu unyunguluka bimpe mu mashi adi ne alkaline. Ino, bakebuludi bapeta amba mu disama dia Tumor Lysis Syndrome, tuselile tua kansere tudi kabidi tuela phosphore wa bungi mu masuba, ne mabue a calcium phosphate adi anyanguka misangu dikumi padi masuba mikale ne alkaline. Kabidi, kuenza bua mashi ikale alkaline kudi kuenza bua calcium wa bungi udi ne ion bua kulamakaja albumine, ne kufikisha ku disama dia hypocalcemic muscle tetany ne disama dia tshimpitshimpi. Lelu, bungi bua bungi bua mâyi a mukele ne Rasburicase bidi binyangakaja aside wa urique kakuyi lutatu nansha kakese kakuyi dijinga dia dienza dia alcaline mu mashi.

Miaba ne buludiki bua mbulamatadi¶

• Société américaine de oncologie clinique (ASCO, États-Unis): Mukenji wa mudimu wa luondapu bua dilama dia disama dia tumor mu babedi bakulumpe ne bana: mikenji ya Cairo-Bishop, diteka dia njiwu, difila dia mâyi mu IV, ne protocole wa Rasburicase.
• Société britannique pour l'hématologie (BSH, Royaume-Uni): Malu adi atangila dikuba ne dilama dia disama dia tumor mu bantu bakole ne bana badi ne disama dia mashi.
• Société européenne pour l’oncologie médicale (ESMO) et l’Association européenne de l’hématologie (EHA): Mibelu ya malu a luondapu idi itangila malu a kansere: disama dia tumor, hyperkalemia, hyperuricémie, ne luondapu lua dipingaja dia mifuba.
• United States Food and Drug Administration (FDA) and European Medicines Agency (EMA): Prescribing Information and Boxed Warning for Rasburicase (Elitek / Fasturtec): G6PD Deficiency Hemolysis, Methemoglobinemia, and Ice-Bath Sample Handling.
• Cancer Institute NSW (eviQ, Australia): Clinical Consensus Protocol: Prevention and Management of Tumor Lysis Syndrome.

Malu adi apetangana¶

• Disama dia tshiadi, rhabdomyolyse, ne disungula dia mashi mafiike
• Disama dia vena cava wa kuulu (SVC) ne disama dia pericardial tamponade dibi
• Methemoglobinemia: mashi a tshikolakola ne bule bua methylene
• Disama dia tshimpitshimpi ne dia tshimpitshimpi diambuluisha dia kumpala
• Nomba ya njiwu bilondeshile ditunga