Rev. Bras. Hematol. Hemoter. vol.39 número4

rev bras hematol hemoter. 2017;39(4):357–359

w w w . r b h h . o r g

Revista

Brasileira

de

Hematologia

e

Hemoterapia

Brazilian

Journal

of

Hematology

and

Hemotherapy

Case

Report

Autoimmune

hemolytic

anemia

and

hyperglobulinemia

leading

to

the

diagnosis

of

multiple

myeloma

Rafael

Lopes

Pacca

,

Jiviane

Beatriz

Cunha

Barretto

da

Silva

,

Kallen

de

Carvalho

e

Souza

_,

_Rebeca

_Barbosa

_Carbinatto

a_{PontíficeUniversidadeCatólicadeCampinas(PUC),Campinas,SP,Brazil}

b_{ClínicaMédicaMedeiros,Campinas,SP,Brazil}

a

r

t

i

c

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e

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n

f

o

Articlehistory:

Received29December2016

Accepted17July2017

Availableonline4September2017

Introduction

Autoimmunehemolyticanemia(AIHA)isaconditioninwhich

self-antibodiesboundtoantigensonthemembranesofred

bloodcellsinitiatetheirdestruction(hemolysis)viathe

com-plementandreticuloendothelialsystems.Multiplemyeloma

(MM),ontheotherhand,ischaracterizedbyaclonal

expan-sion ofplasma cells in bone marrow, causing bone tissue

destruction, renal failure and hematopoietic suppression.

AlthoughtheassociationofMMandanemiaiscommon,AIHA

astheanemicmanifestationofMMisrare.1

Case

report

Herein we report on the case of a 69-year-old, black

patientundertreatmentwithprednisoneandmethotrexate

∗ _{Correspondingauthorat:BancodeSanguedeSertãozinhoRuaEpitácioPessoa1401,Centro,14160-180Sertãozinho,SP,Brazil.}

E-mailaddress:[email protected](R.L.Pacca).

for rheumatoid arthritis over the ten years leading up to

this report. Six years ago, the patient was investigatedby

the hematology department due to an unstable

leukope-nia (2.86×103/␮L), without the involvement of any other

series. Since the beginning of treatment for rheumatoid

arthritis,theleukocyte countofthepatientwascontrolled,

withvariationsbeingattributedtotherheumatoidarthritis

itself.

However, other alterations were found duringa routine

appointment in the Rheumatology Department including

anemia(hemoglobin:7.2g/dL),low hematocrit(22.6%), high

ferritinlevels(732mg/dL),positivedirectcoombstest,and

ele-vatedtotalandindirectbilirubin.Byproteinelectrophoresis,

thetotalproteinwaselevated(11.8g/dL),thealbumin/globulin

ratiowaslow(0.50),thealbuminwaslow(3.94g/dL)andthe

gammaglobulinwashigh(6.25g/dL)withamonoclonal

com-ponent. The patient was referred back to the hematology

department and diagnosed with hypergammaglobulinemia

http://dx.doi.org/10.1016/j.bjhh.2017.07.005

1516-8484/©2017Associac¸ ˜aoBrasileiradeHematologia,HemoterapiaeTerapiaCelular.PublishedbyElsevierEditoraLtda.Thisisan

358

Figure1–Hypercellularbonemarrowattheexpenseof plasmacellinfiltration(Hematoxylinandeosinstain: Magnification:100×).

Figure2–CD138plusplasmocytes.

andAIHAatwhichtimeabonemarrowbiopsywasperformed

thatidentifiedMM(Figures1and2).

Discussion

Normocyticand normochromicanemia, withmultifactorial

origin, such as a red cell series maturation disorder, iron

deficiency,decreasederythropoietinresponseor

erythropoi-etindeficiency, isafrequentfinding inpatientswithMM.2

AIHAisoftenassociatedwithBcelllymphoproliferative

dis-orders.Itcanbeobservedinapproximately8–15%ofpatients

with chronic lymphocytic leukemia (CLL),3 _{as well as in}

otherpathologiessuchasnon-Hodgkinlymphomaincluding

Waldenstrom’smacroglobulinemia,4_{butitsconnectionwith}

MMhasnotyet beenwell elucidated.AreviewbyPirofsky

40years ago showedthatonly4%ofAIHAcaseswere due

toMM.5 _{There are lessthan adozenreported cases,}6 _with

nonebeingBrazilian,butrecentlyaprospectivestudywas

car-riedoutthatshowedthat10.6%ofpatientswithMMhadtheir

pathologycomplicatedbyAHAI.4_{Despitethis,anacceptable}

conclusionhasnotyetbeenreachedaboutthepathogenesis

thatrelatesthesetwoconditions.ThefactthatMMisaBcell

disease,similartoCLL,whichismoreassociatedwithAHAI,

isbeingtakenintoaccounttoconductsomestudies.7_Oneof

thehypothesesisthedevelopmentofautoantibody-producing

clonesagainstthesurfaceantigensofredbloodcells,

origi-natingfromthesignificantimmunedisordercausedbyMM.7

However, no study hasbeen able to provethat the

mono-clonalprotein,ofwhichveryhighlevelsareseeninMM,isthe

antibodyresponsibleforAHAIandthe pathogenesisofthis

progressionisstillunclear.8_{Moreover,duetothelackof}

con-cretedata,thepossibilityofthispathogenesis,thatis,AHAI

asthecause ofMMcannotberuledout.8 _{Theinvolvement}

ofimmunosuppressants,suchasinterferon-alpha,whichare

usedinthetreatmentofMMandotherlymphoproliferative

disorders,mayalsoplayanimportantroleinthedevelopment

ofAHAI,anautoimmunephenomenonsimilartoEvans-like

syndrome.9 _{Thenumberofreportedcasesisextremelylow,}

whichrestrictstheprogressofresearch.

Conclusion

Thiscase describesarare condition, AIHAassociatedwith

MM.Thelownumberofreportedcasesandconsequentlythe

few studieson thisassociation hindersourunderstanding.

However,ongoingstudiesshouldclarifyboththepathogenesis

andtheintimacyoftherelationshipbetweenthetwoentities

inthefuture,therebyallowingearlierdiagnosisandtreatment

thatismoreeffective.Themedicalcommunityshould

con-sider thediagnosis ofAHAIinthe presenceofMM aswell

asvice versa,inordertomakeearlierdiagnosesand

treat-mentsthataremoreeffective.Inaddition,newcasesneedto

bereportedtosupportresearchrelatedtothesetwo

patholo-gies, sothat throughalargeranalysis, sciencecan reacha

coherentandeffectiveconclusion.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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1979;62(12):469–71.

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5.PirofskyB.Clinicalaspectsofautoimmunehemolyticanemia. SeminHematol.1976;13(4):251–65.

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