152ARCHIVESOFDISEASEINCHILDHOODFIG4Smearofalymphnodeshowinglargenumbersofreticulumcellswhichareidenticalwiththex675haemohistiocytesseeninFig2Leishmanx675FIG2Peripheralbloodsmearshowingbinucleateform ID: 859887
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1 LETTERER-SIWE'SSYNDROME:REPORTOFACASEWIT
LETTERER-SIWE'SSYNDROME:REPORTOFACASEWITHUNUSUALPERIPHERALBLOODCHANGESBYN.P.ORCHARD,M.B.,B.S.FromSt.JamesHospital,Balham,London(RECEIVEDFORPUBLICATION,DECEMBER20,1949)In1933SiwelaiddownthecriteriaofthesyndromenowcalledLetterer-Siwedisease,andintheliteraturebeforeandafterthatdatethedifferentfeaturesarediscussedandrelatedtothereportedcases.Thefollowingcaseillustratesinparticulartheperipheralbloodchangeswhichmayoccurinthiscondition,andatableisappendedsummarizingtheclinicalandpathologicalfeaturesof18casesbeginningwithLetterer's(1924)caseandbringingtheseriesuptodate(1950)withthepresentcase.CaseReportAboyaged3jyearswasadmittedonMarch11,1948,withahistoryofsorenessoftherightankle,anorexia,insomnia,andscreaminginthenight.Thetemperaturewasnormal.Thechildwasverypaleandhadpuffyeyes.Firmanddiscretenodesapproximately075cm.indiameterwerepalpableintheneck,especiallyintheposteriortriangle,theaxillae,andthegroins.Thetipofthespleenwaseasilypalpablebuttherewassomedoubtabouttheliverbeingpalpable.Therightanklewasswollenbuttherewasnotendernessandthemove-mentswereneitherlimitednorpainful.Aradiographofthechestshowedadoubtfulhilarshadow;theskull,humeri,pelvis,femora,tibiaeandfibulaewerenormal.BloodCount:Haemoglobin,44%(Haldane).R.B.C.,2,300,000perc.mm.W.B.C.,7,000perc.mm.Differentialwhitecount:neutrophilpolymorphs,4%(280perc.mm.),lymphocytes,95%(6,650perc.mm.),haemohistiocytes,1%(70perc.mm.).Atentativediagnosisofaleukaemiclymphaticleukaemiawasmade.Duringthenextfortnightthetemperaturerangedfrom90°-100°F.andamassbecamepalpableintheleftiliacfossa.28.3.48.Sternalpuncturewasperformed.Inthemarrowfilmshaemohistiocytesdominatedthepicture.Veryfewmaturelymphocytes,granulocytes,andnormo-blastsweretobeseen.29.3.48.Intramuscularpenicillin(20,000unitsfour-hourly)wasstarted.Wassermannreaction++;Kahnreaction,strongpositive.Volmerpatchtestnegativ
2 eafter48hours.Totalserumprotein555g.%.Al
eafter48hours.Totalserumprotein555g.%.Albumin383g.%.Globulin1-72g.%.Albumin/globulinratio2^2:1.Thefather'sandmother'sWassermannandKahnreactionswerenegative.Transfusionof1pintofpackedcells.6.4.48.Wassermann++;Kahn,strongpositive.7.4.48.Penicillindosageincreasedto50,000unitsfour-hourly.11.4.48.WassermannandKahntestsnegative.PaulBunnelltestnegative.22.4.48.Penicillindiscontinued.20.5.48.X-rayexaminationofthehumeri,pelvis,femora,tibiae,andfibulaeshowednoabnormality.26.5.48.Transfusionof1pintofblood.Thespleenwaseasilypalpablebutnottheliver,owingtoguardingintherighthypochondrium.Bypercussiontheliverwasfoundtobeenlargedtwofingerbreadthsbelowthecostalmargin.17.6.48.Biopsyofalymphnodefromtheposteriortriangle.18.6.48.Liverpalpablenow.19.6.48.Childvomitedafewouncesofbrightblood.20.6.48.Occultbloodinthestools.Transfusionof1pintofpackedcells.Patientnowrunningahectictemperature.24.6.48.Reportonsectionofthelymphglanddiagnosedreticulumcellmedullaryreticulosis.30.6.48.Haemoglobinlevelfallingrapidly.4.7.48.Pallornowverymarked,andulcerativeanginawellestablished.Thespleenandliverwerelargerthanbefore.9.7.48.Haemorrhagicgingivitis.11.7.48.Patientdied.TheclinicalfeaturesofthisandotherreportedcasesaresummarizedinTable1.151 152ARCHIVESOFDISEASEINCHILDHOODFIG.4.-Smearofalymphnodeshowinglargenumbersofreticulumcellswhichareidenticalwiththex675.haemohistiocytesseeninFig.2.Leishman.x675.FIG.2.-Peripheralbloodsmearshowingbinucleateformofhaemohistiocytewhichwaspresentinappreciablenumbers.Leishman.x1200.FIG.5.-Lungshowingalveolarseptamarkedlythickenedandinfiltratedbyreticulumcells.Haema-toxylinandeosin.x100.9.4.6-lwRR-Mft.Anr..4w. 153LETJlERER-SIWE'SSYNDROMETABLE1SUMMARYOFBLOODFINDINGSINPRESENTCASEDifferentialWhiteCountoo0C.)R.B.C.Hb.|W.B.C.iCZ+.°AB1I-00Date(millions(Haldane)(millions0perc.mm.)()perc.mm.)ZMarch11,19482-3447,000280-6,650
3 -7014,,,16403,5007035-3,080315,,21,,,1i6
-7014,,,16403,5007035-3,080315,,21,,,1i6383,900195--3,042-66329,,,TransfusionofonepintofpackedcellsApril1,,,3-5684,000360403,52080,,11,,,35685,40086418,,,2*5485,800116I28,,,30644,20084May16,,,2-3506,500130,,21,,,2-2505,20010426,,,TransfusionofonepintofbloodJune9,,,32626,300252-4,158-1,890,18,,,2-4463,100124-2,325-651,20,,,Transfusionofonepintofpackedcells25,,,3-2623,4001102--2,720-578*Theprimitivecellsintheperipheralbloodwereclassedashaemohistiocytes.Theywere15-25,uindiameter,havingalargeroundreticularnucleus,anumbercontainingnucleoli.Thecytoplasmwasabundantandofapaledirtybluecolour(Leishmanstain).Somecellshadcytoplasmicvacuolesandothersfixedpseudopodia.Post-mortemReportExternalAppearance.Averypaleandemaciatedmalechild.Theoralcavityshowedulcerativeanginaandgingivitis.Chest.Oesophagus.Nil.Thymus.Smallandmacroscopicallynormal.Pleura.Leftpleuralsaccontainedonepintofyellowfluid.Larynx.Nil.Lung.Tracheaandbronchinormal.Lungspale:superficialareaofcollapserightlowerlobe.Pericardium.Nil.Heart.Sub-pericardialhaemorrhagesneartheapexandintheauricles.Valves.Nil.Vessels.Nil.Abdomen.Peritoneum.Nil.Stomach.Mucosaverypale.Duodenum.Nil.Bileducts.Patent.Intestines.Peyer'spatchesnotenlarged;solitarylymphfolliclesofthecolonwereprominent.LymphNodes.Thecervical,axillary,pancreatico-splenic,externaliliacandinguinalglandswereredandvariedinsizefrom075-1cm.indiameter.Spleen.Weight,100g.Cutsurfacedarkredandfirm.Liver.Weight,500g.Darkterracottacolour.Structureappearednormal.Pancreas.Nil.Kidneys.Weight,20oz.Capsulesstrippedeasilyleavingasmoothsurface.Thecutsurfacewasverypale.Genito-UrinaryTract.Nil.Brain.Verypaleandmacroscopicallynormal.SpinalCord.Notexamined.Thesternal,vertebral,andfemoralmarrowwasofagreyishpinkcolour. 154ARCHIVESOFDISEASEINCHILDHOODDiscussionThisisacaseofreticulumcellmedullaryteticulosiswithperipheralbloodchanges.T
4 hewhitebloodcountfluctuatedbetweenalowno
hewhitebloodcountfluctuatedbetweenalownormalandfrankleucopenia,withthelymphocyteasthepredominantcell;afairnumberofhaemohistio-cyteswereinvariablypresent.Thereticulumcellsshowedunmistakabledifferentiationintwodirec-tions,namely,haemicandhistiocytic.Withoutadoubtthehaemohistiocytesintheperipheralbloodandinthemarrowweresimilartotheproliferatingreticulumcellsinthelymphglands,spleen,liver,lungs,andkidney.Theyweremedullaryreticulumcellswithatendency,asfarasthenuclearstructurewasconcerned,towardshaemicdifferentiation.Definiteevidenceofdifferentiationtowardshistio-cyteswastobeseenintheliver,spleen,lungs,kidney,and,toalesserextent,inthelymphnodes.ApartfromafewdeparturesfromtheacceptedpictureoftheLetterer-Siwesyndrome(namely,thepresenceofhaemohistiocytesintheperipheralblood,andtheabsenceofpurpuraandosseouschanges)thecasereportedsatisfiesthecriterialaiddownbySiwe.TheWassermannandKahnreactionswerestronglypositiveontwooccasionsbutnegativeonthethird.Thisnodoubtwasduetothechangeintheplasmaglobulin.SummaryAnunusualcaseofreticulumcellmedullaryreticulosisdisplayingthefeaturesoftheLetterer-Siwesyndromeisdescribed.AtabularreviewofthecasesreportedintheliteratureispresentedintheAppendix.MythanksareduetoDr.B.Barlingforpermissiontopublishthecase,andtoDr.A.H.T.Robb-Smithforhishelpfulcriticismandencouragement.REFERENCESAbt,A.F.,andDenenholz,E.J.(1936).Amer.J.Dis.Child.,51,499.Akiba,R.(1926).VirchowsArch.,260,262.Creveld,S.van,andTerPoorten,F.H.(1935).Arch.Dis.Childh.,10,125.Foot,N.C.,andOlcott,C.T.(1934).Amer.J.Path.,10,81.Gittins,R.(1933).Arch.Dis.Childh.,8,367.Guizetti,H.U.(1931).VirchowsArch.,282,194.Klostermeyer,W.(1934).Beitr.path.Anat.,93,1.Krahn,H.(1926).Dtsch.Arch.klin.Med.,152,179.Letterer,E.(1924).Z.Pathol.,30,377.Podvinec,E.,andTerplan,K.(1931).Arch.Kinder-heilk.,93,40.Roussy,G..andOberling,C.(1934).Wien.med.Wschr.,84,
5 407.Sherman,I.(1929).Arch.Path.,7,78.Siw
407.Sherman,I.(1929).Arch.Path.,7,78.Siwe,S.A.(1933).Z.Kinderheilk.,55,212.Uher,V.(1933).VirchowsArch.,289,504. LETJTERER-SIWE'SSYNDROME155A'PPENDIXANALYSISOFFEATURESOFREPORTEDANDPRESENTCASESReticulosisofClinicalFeaturesOrgansAge"1coto~OAuthorofPentngOsseousChangePatientSigns-e&mU)-Letterer(1924)6mths.Purpura11Iwks.+-+-+I+4++Noneclinically++++++sepsisAkiba(1926)10Omths.Purpura6wks.+++±?+_+++,,,++?+++JsepsissKrahn(1926)5yearsSepsis6mths.+++S+++,,++++++'Sherman(1929)ildaysJaundice4days+--+--++,,,?+++++erysipelasIGuizetti(1931)3mths.Cough,21days+-++-?++++Cysticchangesinthe+++++++±1+fever,righthumerusonepistaxisx-rayexaminationPodvinecandTerplan1yearFever14days+++--+++++Noneclinically++++++++(1931)Uher(1933)1yearsSorethroat,*17days+++-S+++,,9,,++++++++feverSiwe(1933)16mths.Swellingof4mths.±+-S+++ICysticchangesinthe±++++±±theleftlegleftfibulaonx-rayexaminationCGittins(1933)(a)23mths.Anaemia24,mths.+-:++±+S-++Noneclinically++4+±±+?(b)21lmths.Anaemia3wks.+-+i+++++-,,+±++?abscesses(C)1yearAnaemia11days?--+++++±++Slightx-raychanges+-+++?(d)17wka.Anaemia4mths.--S+++_+±+Nonedcinically+!++&++Klostermeyer(1934)13mtha.Swellingof7wks.++-+A++±++,,++++?thefaceThs^stantiOkntt95sasPo.qcWr,,ira,,~11--mi,(1934)otitism'e`diaRoussyandOberling8mths.Purpura1mth.+-++S+(1934)vanCreveldand4mths.Otitismedia8wks.+---S+TerPoorten(1935)AbtandDenenholz2yearsTumourof4imths.+-+±±theskull-revealedra-re-fa-ction++++++±ofthepelvis,upperendoftherightfemur.Osteoporosis,ofthespineatfirstsuggestedPott'sdis-ease+++Noneclinically+++++++++++Cysticchangesinthe+++++++skull,headoftherighthumerusandlowerendoftherightfemur±±±±+Pulsatinattumourof-l-±±±±++theskull.Onx-rayexaminationacysticIareawasqannarent±±i-4-±?=Notmentioned.S=Slight.A=agranulocytosis.Swellingof4mths.++IS++A+++INoneclinically+++~++rightankde,IIanorexia,insomnia6..i--1-1----?=Notmentione