Case report Hydroxyurea therapy for management of secondary erythrocytosis in cyanotic congenital heart disease Urike M. Reiss," Pamela Bensimhon? Sherri A. Zimmerman,? and Russell E. Ware? * cw Heng Dene rai Ds Caro aa Cra Dahan ath Ca Secondary erythrocytosis in eyanotle congenital heart disease (CCHD) causes. substantial morbllty because of complications of hyperviscosily, Including stoke and chronic end organ damage. Phlebotomy Provides temporary Improvement but leads to on deficency and can actualy Increase blood viscosity. We Sescibe the successful use of hydroxyurea (hydroxycarbamide) in four patients with uncorrected CCHD ‘and symptomatic secondary erythrocytosis. In all patients, hydroxyurea improved symptoms of hypervis- Cosi. Substantial decreases in ‘mean corpuscular volume (MCV) and mean eorpust Inthe culating hemoglobin concentration. Two patlents experienced t linlch promptly resolved with dose reduction of hydroxyurea. Hydroyxurea p sty from secondary erythrocytes inpatients with CCHD, while Therapeutic approach te reduce hyper ‘ed blood call (RBC) count were observed slong with increases inthe hemoglobin (ICH), ea a preserving oxygen carrying capacity and avoiding ron depletion by phlebotomy. Am. Hematol 82740" 443, 2007. © 2007 Wiley-Liss ne Introduetion ‘Compensatory secondary erthoaytass Is a common ‘icing patents with conic fypoxeria because of cya rate eangenl Rea” dsease (CCMD}. In tha cricl st fing ntacaraae egrttetet shunting alow. desaturated omogebin ‘o enlar the altel eeaton, Which ten ‘Gade to dinning delvary cf oxygen tothe tosuas. In ‘espensa, a phystlegieal ner Fgher hemoglobin concentration, but eventual leads to signs and symptoms ot mypervscosty such as headaches, tone pan. fapratory dress, wansent chem tac, Soke, and chronic end organ damage [1-3]. In aston, yparlecosty can desrasee cards Sup and Amity (Gn -cetvery. whlch further stmaates.entvocyoeis an ‘Eblenes 4 vious cya Ae tepore! lv 8 rasan survey, ‘scar adverse events remain a sastanta cause of mot: iy congedlher Sotse, spain paers pallets wha cyanotic detect higher Than wih ny ofr arias detact “To decrease, hyperdscosty,itamitent phlebotomy is ctton permed to mata a haratoctt of lass th iovel tat usualy. proves adequate onygen cand apacty. However the approach can have sefous conse fqlonces including Inadequate angen dalvery and sjpto- ‘hate hypoxemia, a2 wall a eft from acute changes 9 ‘ood Wolume and vacos iy, whieh may. paradoxcaly ‘Reraase the rak of stoke or shor end organ damages pendent of the hematott (6.6, Repeated prisbotomy may Feu in on detconcy leading 1 nereased get WEA ‘ed cals, wich furtnet Ineeases the nak et syrofomate yperiscosty f-—11) ycronnures (hydorycabamide) is an S-phase spect chemathorapee agent ta Ins rlborucletise reductase, land thus etereres wth te production of DNA precursors (© 2007 Wiley-Liss, Ine. Ano Sent Hess 140 ‘que for cll ropllcaon and nuclear maturation, Hysox. Jules‘nauced rucearcyoplasmle ayynetwony Io asso Banlad by & proound macrocyosis and prolonged pro- Sicon of cyepatmle hamogienn [12-14) Hyataxyurea ‘horapy fo sacanaary eryivecyosls Should therefore ower ood iscosty by reducing RBC count and hemafort ‘ough marrow Svppresson, hla srnuaneoushy prov ing axygercarrying capacty per eryrocyte by ineressing ‘he MOV and NCH. The use ot fyaraxyuta In COHD has boon previously deacrbed 216,16} but was not includ Ir two cant reviews of the management of sacondary eryvocyisis n OCHO [17.18) We now report the suc esc dae of hyconyures in Your paints wih uncorrected {ECHO and symptomate. secondary ‘ve the pathophysiology a6 wat ‘sof his theepeteapproce. ‘case Reports ou patents were rela for hematologeal consutaton because of sympromate secondary arytecjtoss (Tabs Each patient had an undetiyng hagnsis of CCHD, ncling ‘otalogy of Falot win pulmonary esa touapid stent, ingle ight venti, or eraventncuar canal detec Al had Ineompiete repair duting early childhood and remained with portent invacardac mango Bcod subsequent deve {ig secondary enthrooyfons, On prassnatn. the pants wove 16, 18,22 and 2 years ld (8 mals, 1 female). To artpoace : UthaRat, Capra of Hay Sue ‘nd seu ferpa Sah Cao orp, aes Gent an aera le Bar sonaynaxcas