Rev. Bras. Reumatol. vol.57 número5

r e v b r a s r e u m a t o l . 2017;57(5):472–474

ww w . r e u m a t o l o g i a . c o m . b r

REVISTA

BRASILEIRA

DE

REUMATOLOGIA

Case

report

Coexistence

of

hypertrophic

osteoarthropathy

and

myelofibrosis

夽

Coexistência

de

osteoartropatia

hipertrófica

e

mielofibrose

Bayram

Kelle

,

Fatih

Yıldız

,

Semra

Paydas

,

Emine

Kılıc

Bagır

,

Melek

Ergin

_,

_Erkan

_Kozanoglu

a_{CukurovaUniversity,FacultyofMedicine,DepartmentofPhysicalMedicineandRehabilitation,Adana,Turkey}

b_{CukurovaUniversity,FacultyofMedicine,DepartmentofRheumatology,Adana,Turkey}

c_{CukurovaUniversity,FacultyofMedicine,DepartmentofOncology,Adana,Turkey}

d_{CukurovaUniversity,FacultyofMedicine,DepartmentofPathology,Adana,Turkey}

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Articlehistory:

Received1September2014 Accepted2November2014 Availableonline28January2015

Introduction

Hypertrophicosteoarthropathy(HOA)isaconditionpresented byarthralgia/arthritis,clubbing,andperiostealproliferation (periostitis)inlongbones.Itisclassifiedasprimary(hereditary oridiopathic)andsecondary.PrimaryHOAisarareand hered-itaryconditionobservedmainlyinchildrenandadolescents.1 SecondaryHOAmaybeseeninthevarioussystemicdisorders includinginflammatoryandmaligndiseases.Clinicalfindings developasaresultofanincreaseinperipheralbloodflowas wellasabnormalfibroblastactivityandproliferation.2–4

Myelofibrosisisachronicmyeloproliferativedisease char-acterizedbyclonalexpansionofanabnormalhematopoietic stem/progenitorcell.5_{Thereareafewcasereportsregarding}

夽

ThisstudywasoriginatedinCukurovaUniversity,FacultyofMedicine,DepartmentofPhysicalMedicineandRehabilitation,Adana, Turkey.

∗ _{Correspondingauthor.}

E-mail:bayramkelle@yahoo.com(B.Kelle).

thecoexistenceofmyelofibrosiswithHOA.Herewepresenta caseofHOAcoexistedwithmyelofibrosis.

Case

report

Sixty-fiveyearsoldmaleadmittedtoPhysicalMedicineand RehabilitationOutpatientClinicduetoincreasedpainatboth lowerextremitiesforthepreceedingyear.Hewasdiagnosed myelofibrosisasaresultaftermedicalhistory,routineblood tests and bone marrow biopsy 6 years ago. Bone marrow aspiration wasdry,andexaminationofthebiopsy revealed a hypoplastic marrow with myelofibrosis (Fig. 1A and B). Patientreceivedinterferon(IFN)alfa2Aduring2yearsandhis bonemarrowwasinremissionafterthistreatment.Although

http://dx.doi.org/10.1016/j.rbre.2014.11.004

r e v b r a s r e u m a t o l . 2017;57(5):472–474

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Fig.1–Bonemarrowbiopsyshowsreticulin-typemyelofibrosis(hematoxylinandeosin,100×).Bonemarrowbiopsyshows

reticulin-typemyelofibrosis(reticulin,100×).

patient’s painhasincreasedwhilemoving, it hasalso per-sistedatrest.Patienthadbenefitedfromanalgesicmedication initiallybuthestatedthathispainincreasedovertime,and notresolvedwithanalgesics.Onthe physicalexamination, patient’sgeneralstatuswaswell.Bloodpressure was mea-suredas125/75mmHg,andpulseratewas78min–1_.Clubbing was observed at both hands. Both tibias were painful by percussion, and tibial margins were palpated as irregular. Althoughpainwaspresentatanklesandknees,no temper-aturerise or synovitiswere detected. Range ofmotion for bilateralhip,kneeandanklejointswerewithinthenormal limits.

Conventional X-ray of the cruris region demonstrated periostealreactionatbothtibialbonewhichismore promi-nentontheleftside(Fig.2).Wholebodybonescintigraphy demonstratedincreaseduptakeatdistalpartoflefttibia.In laboratoryanalysis,hemoglobin,whitebloodcellandplatelet countswerewithinnormallimits,whileMCVwasdecreased [69.5fL–(N:80–97–)].Peripheralbloodsmearwasassessedas

Fig.2–Periostealreaction(periostitis)atthetibialmargin (blackarrow).

normal.Therewasnotanincreaseinacutephasereactants. Erythrocytesedimentationratewas20mm/h(N:0–15)andCRP waswithinnormalrange[<0.5ng/L(N:0–0.8)].Parathyroid hor-mone,calciumand25-hydroxyvitaminD3levelswerewithin normal limits.SerumIL-6 and TNFalfa levelswere within normalrange.

Diagnosis was madeas secondary HOAassociatedwith myelofibrosis. Patient was recommended to receive oral meloxicam15mgdaily.Becauseoftheincompleteresponse aftersevendays,colchicine1mg/daywasaddedtothe treat-ment,andsignificantdecreaseofpainwasdetectedafterone week.

Discussion

HOAisaclinicalsyndromecharacterizedbydigitalclubbing andperiostalproliferationespeciallyinthetibialbone.1_HOA includesskinmanifestationssuchasthickenedskinonface and scalp, and coarse facial features along with clubbing, periostosis,acroosteolysis,andpainfuljointmotion.6

HOAmaybeassociatedwithvariousdisorders including intrathoracicmalignancies,cyanoticheartdiseases, gastroin-testinaltumorsandinflammatoryboweldiseases.1_Oneofthe rarecausesofsecondaryHOAismyelofibrosis.

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ofthelimitationsofthiscasereportwasthelackoflaboratory testsincludingPDGF,TGFandVEGF.Nevertheless, TNF-alfa andIL-6levelswerenegativeinourpatient.

HOAassociatedwithmyelofibrosisisobservedrarelyand alimitednumberofcasereportsarepresentedinthe litera-ture.Yuetal.presentedtwomalewithHOAassociatedwith myelofibrosis.Theysuggestedthatperiostitis,when associ-atedwithfeverandbonepain,isindicativeofmoreaggressive disease.10 _{In several case reports of HOA associated with} myelofibrosisithasbeenreportedthattheacutephase reac-tantswerehigher.2,3,7_{Ourpatienthadnotfeverandtherewas} noevidenceofincreasedacutephaseproteins.Thesepoints maybeassociatedwiththemilderdiseasecourseofourcase. ThereisnoevidenceintheliteratureregardingthatIFN causesclinicallyovertHOA.ButIFNtherapymaycause club-bing in some patients. There was only a published report consistingtwopatientswhohadclubbingassociatedwiththe use ofIFN treatment.11 _{Itwas reportedthat IFNtreatment} causesabnormalfibroblastactivationandthisconditionmay bethecauseclubbing.Inthiscasereporttwopatientswere presentedwhoreceivedIFNalfa2AforhepatitisCvirus.Digital clubbingwasdevelopedafterthe2ndand4thmonthsof med-icationinthesepatients,respectively.Incontrast,ourpatient wasdevelopeddigitalclubbing andtibial bonepainafter2 yearsofmedication.Nevertheless,therewasaperiosteal reac-tioninbothtibialbonesinadditiontoclubbinginthecurrent casewhichprovidedthediagnosisofHOA.

Thereisnoconsensusonthestandardtreatmentregimen ofHOA.Afavorableresulttothecombination ofcolchicine andmeloxicamwasobservedinthepresentcase.Inthe liter-ature,ithasbeenreportedthatpamidronatehasbeenfound tobeeffectiveinosteoarthropatiesnotrespondingto nonste-roidalanti-inflammatorydrugs.Octreotidetreatmenthasalso yieldedsimilarresults.12

Inconclusion, patients who had diffusejoint pain with unknowncauseshouldbecheckedforcomorbidconditions andadditionaldiseases.Inaddition,HOAshouldbesuggestive incasespresentedwithatypicallocalizedpainandclubbing alreadywhohadmyelofibrosiseitherinactiveorremission period.However,itshouldbenotedthatallclinicalfindings may not be observed in cases of secondary or associated HOA.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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