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Thrombocythemia as a cause of oral hemorrhage
Thrombocythemia as a cause of oral hemorrhage X. ,l. Yogrel, R.D.S., X3., St. ,lstrph, AVorth Tl’tr1e.s II. 31. ST‘QJIXY
C’h.B., F.D.S.K.C.S.,”
IIOSI’IT.\L
‘~h~o~~~l~ocytl~en~ia is characterized by an increase in platelet numbers couplrd vitll :Ilmormxl function. Three cases diagnosed after referral to WI oral surgeon :IW pwwntetl. In each case the reason for referral concerned csccssivt~ drntal hcrnorrllap~. Tllronl~,ocytllc,r~li~ sl~ould lw included in the differential diagnosis in cases of ord Ilcnlorrllngc~.
T
hrombocythemia is a primary disorder of marrow which results in thrombocytosis, an increase in the number of circulating platelets. Most laboratories acccl,t. a normal platelet count as being between 150,000 and 350,000 per cubic millimeter. Thrombocytosis can be diviclcd into primary (idiopathic) and secontlilr,V (reactive) forms. The idiopathic form is thrombocythemia, whereas the reactive form can be secondary to many conditions, including infections, trauma, cmeratiw procedures (particularly splenectomy), anemia, Gushing’s disease, rheumatoid arthritis, and malignant disease. The clinical and laboratory differences between the two states are summarized in Table I. Patients with primary thrombocythemia may be of either sex and are usually middle-aped. Symptoms, when present, include bleeding, particularly from mucous m~ml~r~n~~, and bruising, but rarely petechiae.l The tourniquet test is ilormally ncgative.2 Thrombosis is also common, particularly in the splenic vessels. 8pIrnomcga1y alld hepatomegaly commonly occur. The bleeding time is freqitrntly elevated, but the clotting time is normal. Examination of a peripheral blood film sl~ows an increased number of platelets, often irregularly distended, with some giant forms. A lcukocytosis is common. Polycythemia or anemia can OWLII~, ililt these conditions may alternate with each other. An examination of the hJzlc marrow n-ill normally show a hyperplastic condition, with hyperplasia of the m>-eloitl and crythroid series. There may also be areas of fibrosis. The megaknryocytcs often have a normal appearance. ‘,Scnior
Registrar
in Oral Surgery.
535
Table
I
-
-_-. Reactive
Hemorrhage or thrombosis Duration Spienomegal! Platelet count Bleeding time l’latcir~ morpholog)
rhromboc~tosl.\
Rare Transitory ho Karel) wt‘r I .0(H) * 10” I Normal Normul Yormal __-.
-I-
7’hrornbocrrhrmra
Common Lasting Present In 80!~ ul us29 Commonly over 1.000 1 IO’ I Orten raised 4bnormal I- rcquentll elevated
DISCUSSION
1(liopathic thronil~oc~tlicntia is gencrall~- considered to bc a m,vtloprolifcrativ~~ tlisortlcr, together with polycythemia ant1 myelofibrosis, and some element. of all thrcscl disorders is often present, as (Icmonstratetl in Cast 1. The true incidence of the tliwasc is unl;no~-II but it is the rarest of the m!-cloproliferative disorders; in 1his arca, whcrc there is onl,v one l~cmatolog~ laboratory for a population of a !luilrtclv of a million psoplt~, there have been only two new cases in the last 2 ~-c;lIX. The tlisorder is not llcrctlital~y,4 but a familial raw has been reported.” Some 1)hpsiologic aspects of the abnormal platelet fundion haw been elwitlatc~cl. Thaw is abnormal platelet aggregation in response to several aggregating agents.‘, The prrsencc of an excessi\-c number of platelets also seems to prevent tllronil~o~~lusti~i formation.7 Platelet factor 3 has been variably reported to lw cithw 110rlni~l or dccrcasedC~ ‘I‘lironil~o~~tlicmia has been considered to bc a benign condition, but death (‘a11 owur from hemorrhage ant1 thrombosis. Thcrc can also be progression to myolofil)rosis, n-hich is itself a chronic contlition but which frequently tcrminat es in nlarrow h,vpoplasia or leukemia.” ( )pillions tliffcr concerning the 1reatmcnt of thrombocythemia. In asympl omi~ti~ (eases,some authorities tlo not advocate active treatment, as in the first C~SC prcwwttvl, whc~rcas others atlvisc prophylactic trtatmcnt. Synipt,omatic CaSc’S alw;rys rcquiw treatment. The treatment rccommentletl iwludes radioactirc
C’h.B., F.D.S.K.C.S.,”
IIOSI’IT.\L
‘~h~o~~~l~ocytl~en~ia is characterized by an increase in platelet numbers couplrd vitll :Ilmormxl function. Three cases diagnosed after referral to WI oral surgeon :IW pwwntetl. In each case the reason for referral concerned csccssivt~ drntal hcrnorrllap~. Tllronl~,ocytllc,r~li~ sl~ould lw included in the differential diagnosis in cases of ord Ilcnlorrllngc~.
T
hrombocythemia is a primary disorder of marrow which results in thrombocytosis, an increase in the number of circulating platelets. Most laboratories acccl,t. a normal platelet count as being between 150,000 and 350,000 per cubic millimeter. Thrombocytosis can be diviclcd into primary (idiopathic) and secontlilr,V (reactive) forms. The idiopathic form is thrombocythemia, whereas the reactive form can be secondary to many conditions, including infections, trauma, cmeratiw procedures (particularly splenectomy), anemia, Gushing’s disease, rheumatoid arthritis, and malignant disease. The clinical and laboratory differences between the two states are summarized in Table I. Patients with primary thrombocythemia may be of either sex and are usually middle-aped. Symptoms, when present, include bleeding, particularly from mucous m~ml~r~n~~, and bruising, but rarely petechiae.l The tourniquet test is ilormally ncgative.2 Thrombosis is also common, particularly in the splenic vessels. 8pIrnomcga1y alld hepatomegaly commonly occur. The bleeding time is freqitrntly elevated, but the clotting time is normal. Examination of a peripheral blood film sl~ows an increased number of platelets, often irregularly distended, with some giant forms. A lcukocytosis is common. Polycythemia or anemia can OWLII~, ililt these conditions may alternate with each other. An examination of the hJzlc marrow n-ill normally show a hyperplastic condition, with hyperplasia of the m>-eloitl and crythroid series. There may also be areas of fibrosis. The megaknryocytcs often have a normal appearance. ‘,Scnior
Registrar
in Oral Surgery.
535
Table
I
-
-_-. Reactive
Hemorrhage or thrombosis Duration Spienomegal! Platelet count Bleeding time l’latcir~ morpholog)
rhromboc~tosl.\
Rare Transitory ho Karel) wt‘r I .0(H) * 10” I Normal Normul Yormal __-.
-I-
7’hrornbocrrhrmra
Common Lasting Present In 80!~ ul us29 Commonly over 1.000 1 IO’ I Orten raised 4bnormal I- rcquentll elevated
DISCUSSION
1(liopathic thronil~oc~tlicntia is gencrall~- considered to bc a m,vtloprolifcrativ~~ tlisortlcr, together with polycythemia ant1 myelofibrosis, and some element. of all thrcscl disorders is often present, as (Icmonstratetl in Cast 1. The true incidence of the tliwasc is unl;no~-II but it is the rarest of the m!-cloproliferative disorders; in 1his arca, whcrc there is onl,v one l~cmatolog~ laboratory for a population of a !luilrtclv of a million psoplt~, there have been only two new cases in the last 2 ~-c;lIX. The tlisorder is not llcrctlital~y,4 but a familial raw has been reported.” Some 1)hpsiologic aspects of the abnormal platelet fundion haw been elwitlatc~cl. Thaw is abnormal platelet aggregation in response to several aggregating agents.‘, The prrsencc of an excessi\-c number of platelets also seems to prevent tllronil~o~~lusti~i formation.7 Platelet factor 3 has been variably reported to lw cithw 110rlni~l or dccrcasedC~ ‘I‘lironil~o~~tlicmia has been considered to bc a benign condition, but death (‘a11 owur from hemorrhage ant1 thrombosis. Thcrc can also be progression to myolofil)rosis, n-hich is itself a chronic contlition but which frequently tcrminat es in nlarrow h,vpoplasia or leukemia.” ( )pillions tliffcr concerning the 1reatmcnt of thrombocythemia. In asympl omi~ti~ (eases,some authorities tlo not advocate active treatment, as in the first C~SC prcwwttvl, whc~rcas others atlvisc prophylactic trtatmcnt. Synipt,omatic CaSc’S alw;rys rcquiw treatment. The treatment rccommentletl iwludes radioactirc