Biklonalna gamapatija (IgD mijelom i bolest lakih lanaca) inicijalno ispoljena bubrežnom insuficijencijom

  • Violeta Rabrenović Clinic of Nephrology, Military Medical Academy, Belgrade, Serbia; Medical Faculty of the Military Medical Academy, University of Defense, Belgrade, Serbia
  • Zoran Mijušković Medical Faculty of the Military Medical Academy, University of Defense, Belgrade, Serbia; Institute of Medical Biochemistry, Military Medical Academy, Belgrade, Serbia
  • Slobodan Marjanović Medical Faculty of the Military Medical Academy, University of Defense, Belgrade, Serbia; Clinic of Hematology, Military Medical Academy, Belgrade, Serbia
  • Milorad Rabrenović Center of Hyperbaric Medicine, Military Medical Academy, Belgrade, Serbia
  • Dragan Jovanović Clinic of Nephrology, Military Medical Academy, Belgrade, Serbia; Medical Faculty of the Military Medical Academy, University of Defense, Belgrade, Serbia
  • Svetlana Antić Clinic of Nephrology, Military Medical Academy, Belgrade, Serbia
  • Ljiljana Ignjatović Clinic of Nephrology, Military Medical Academy, Belgrade, Serbia
  • Milica Petrović Clinic of Nephrology, Military Medical Academy, Belgrade, Serbia
  • Dejan Pilčević Clinic of Nephrology, Military Medical Academy, Belgrade, Serbia; Medical Faculty of the Military Medical Academy, University of Defense, Belgrade, Serbia
DOI: https://doi.org/10.2298/5482
Ključne reči: kidney failure, acute||, ||bubreg, akutna insuficijencija, multiple myeloma||, ||multipli mijelom, diagnosis, differential||, ||dijagnoza, diferencijalna, biopsy||, ||biopsija, histological techniques||, ||histološke tehnike.,

Sažetak


Uvod. Imunoglobulin D (IgD) mijelom veoma je retko oboljenje jer čini oko 2% multiplih mijeloma, a još ređe se opisuje njegova udružena pojava sa drugim mijelomom u okviru biklonalne gamapatije. Prikazan je bolesnik sa IgD mijelomom i bolešću lakih lanaca koje su se inicijalno manifestovale bubrežnom insuficijencijom. Prikaz bolesnika. Kod muškarca, starog 37 godina, mesec dana pre hospitalizacije javili su se malaksalost, zamaranje i smanjeno mokrenje. U laboratorijskim analizama zapažena je azotemija, pa je plasiran dijalizni kateter i započeto je sa hemodijalizama. Nakon toga hospitalizovan je radi daljeg ispitivanja. Pri prijemu u objektivnom pregledu zapaženo je izraženo bledilo uz hepatosplenomegaliju i arterijsku hipertenziju (170/95 mmHg). U laboratorijskim analizama sedimentacija je bila ubrzana – 122 mm/h, bio je izražen anemijski sindrom (Hb 71 g/L), i bubrežna insuficijencija dijaliznog ranga: kreatinin 1 408 μmol/L, ureja 31,7 mmol/L. U elektroforezi proteina u serumu zapažene su dve M komponente – IgD lambda i slobodni laki lanci lambda tipa. Registrovana je proteinurija nefrotskog ranga (5,4 g/24 h), a elektroforezom proteina u urinu zapažene su 2 M komponente – masivna u α 2 frakciji (71%); diskretna (7%) u β frakciji; beta 2M u serumu 110 mg/L, a u urinu 1,8 mg/L – izuzetno povišeni; IgL kapa/lambda indeks 1:13 (normalna vrednost odnosa 2:1). Nalaz je ukazivao na dvostruku mijelomsku bolest: IgD mijelom i Bence Jones lambda mijelom. Učinjenom biopsijom kosti dokazan je IgD mijelom lambda – 100% infiltracija medule, pretežno plazmoblastima. Nastavljeno je lečenje hemodijalizama tri puta nedeljno uz hemioterapiju po protokolu: bortezomib, doksorubicin, deksametazon. Nakon četiri ciklusa hemioterapije došlo je do smanjenja koncentracije IgD, lakih lanaca-λ, smanjenja proteinurije (1,03 g/24 h). Broj hemodijaliza je takođe smanjen na jednu nedeljno. Nakon šest meseci od započinjanja lečenja učinjena je autologna transplantacija matičnih ćelija koštane srži. U periodu praćenja od dve godine kod prikazanog bolesnika osnovna bolest bila je u remisiji. Zaključak. Prikazana retka dvostruka mijelomska bolest koja se ispoljila inicijalno bubrežnom insuficijencijom, ističe značaj pažljivog ispitivanja i timskog rada kojim se može izmeniti tok ovog ozbiljnog oboljenja.

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Objavljeno
2015/07/08
Broj časopisa
Vol. 72 Br. 2 (2015)
Rubrika
Prikaz bolesnika