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From www.bloodjournal.org by guest on June 17, 2017. For personal use only. Pseudotumor of Hemophilia with R. By LTHOUGH tions also may the PTC SJLBER Deficiency W. AND hemarthroses in a Patient of B. CHRISTENSEN hemophilia are osteolytic lesions are generally accompanied and usually, therefore, are called pseudotumors and roentgenologic manifestations may be malignant in osteolytic the tumors. literature. following This case report A total rare of a patient L.W.:This 27-year-old the for patient had Slight hip. a period mass appeared in the thigh. right the PTC the other 3, of 5, knee symptoms the patient had trauma to profuse for 10 hemarthroses. at the treatment the Because of referred About sustained of However, three of course this, but months the of 10. severe Five had years and and the the over the the right a painless this gradually anterior aspect which lasted bleeding into A of blood In the and and carefully maternal joint. of transfusions hematuria occupation the 2 weeks. the hemarthrosis bleeding. of on of period. gingival a cousin General admission, accident, multiple bouts to spontaneously months by later, Lake region extractions. a sedentary uncle the nine this dental repeated sought grand in subsided after followed epistaxis noted prior epistaxis was after Salt to following during the year this developed knee noted had he A trauma a spontaneous left age of minor noted suffered patient exercise. been to one trauma, Numbness was the the the the bleeding years, physical was mass. had recorded deficiency. draftsman site In been illustrated REPORT a watermelon. developed following strenuous flank. of minor required the No of age right were that the bleeding Such tissue swelling The clinical from those of have is abdominal and at weeks. right to later, the a staircase four the an noted about size age year At on was in increased At fallen of male regarding swelling over One white consultation cases complication with manifesta- abnormal articulations. massive soft of hemophilia. indistinguishable 18 such serious that the by of only but CASE Hospital well-known of this disease, it is not commonly realized cause bone destruction in sites remote from side course of numerous avoided were known hemophiliacs. Physical vital examination signs of the not of A was as No the joints and as obese mass discrete and posteriorly into was The revealed male, bulging tenderness hemorrhage. was not otherwise an large, firm well warm. accompanying tion It ligament was revealed normal. abdomen. inguinal skin were mass limitation who was was neither readily extended the region from the the right of elicited on was readily palpable of abduction pressure. of pale on costa! rectal right icteric. the The side was no to the overlying evidence examination. hip. The right margin flank. There on the nor apparent of Examina- Physical examination remarkable. From the Departments Salt Lake City, Utah. of Niedicine and Radiology, University of Utah, College of Medi- cine, One of the authors (R.S.) is a Trainee, National Cancer Institute, Public Health Service. The authors acknowledge Maxwell Wintrobe, Ceorge with E. sincere Cartwright appreciation and the Russell S. advice Jones paper. Submitted July 7, 1958; accepted for publication 584 July 25, 1958. in and assistance the preparation of of Drs. this From www.bloodjournal.org by guest on June 17, 2017. For personal use only. PSEUDOTUMOR Fic. The volume 7850 per of platelets was 216,000 per The not cu.mm. The to Additional defect of sky units; mg. per X-ray the The serum hundred ml. of intact. the Only major demonstrable. soft new, mass The tissue gested either to mass. that by to PA film the extensive a neoplasm, from of the chest soft such as ilium and tissue osteogenic of ilium calcification apparent a skeletal and sarcoma, survey the in of pelvis. bone a all the coagulation 5.5 the not were no longer except pos- induced in body remarkable. had of well-defined and main hematoma. 3.5 remained been medial of of It The some its fragments destruction Bodan- destruction present within large the directions were irregular normal was fairly ilium was of phosphorus body Some were or the 15 hours. defect structure lobulated, fragments of serum bony of a large, the The irregular this portion of the were mass of with flecks midline the extensive, lateral body and two addition correct was was after phosphatase ml. showed discrete Irregular the of the the not and ) Lee-\Vhite) the was Cronkite abnonnal. did count erythrocytes good after alkaline portions and was serum hundred associated displaced formation. remnants crest ) ( time normal The 1) blood the and was Douglas inferior of Schneiderman became per 1956. white Clotting patient’s (fig. was clearly extended be the extended had bone which presumed which this and defect and test The retraction deficiency. hips ml. 3, morphology negative. clot mg medial of bony mass periosteal portion, and portion This tissue teriorly. that 10.8 the was the PTC calcium abdomen ilium. The showed 100 ( Brecher, ( Biggs since October The Qualitative known pelvis, ml.! count test serum with 42 test ). of normal. platelet tourniquet studies the was was minutes patient’s a patient right cells generation the serum. red The 12 thromboplastin localized film differential unusual. ( control, minutes 1.-Anteroposterior packed cu.mm. 585 HEMOPHILIA OF bone the was produced irregularity soft sug- From www.bloodjournal.org by guest on June 17, 2017. For personal use only. 586 SILBER of tile of the defect, OSSCOUS mass periosteal and the consideration. The he be hospitalized, An ordinary A small, spine. forth. The men residual at seen. only to The On its and 4, The continued tempt to large plasma plasma to by size. was also I)eterioration and he cultured became disoriented. improve the following death despite the the with that this cavity in the day the tumor. speci- elements the The hematoma an effusion which patient had outcome, its original of pseudotumor ml. of anemia was continued to decreased Despite continuous 1957. The same simultaneously. patient became These symptoms grand confused did mal not seizures patient’s abdominal of hospitalization. size. This and mass had At decrease repeatedly at- repeated, slowly months had an 750 His appeared several the of May. February The four average course. in aureus In of wound downhill the size. however, septicemia General staph. regime to mass, appeared. Lake in operative flank last Salt a December edema 1/3 the disease, complication. the about of unchanged and fatal during was the the size formed a hemolytic received from pleural 23, Despite in swelling fact May of had emptied the occurred and refilled blood. Radiographs ment in obtained appearance. constitution in day. decreased of after On patient a desultory dyspnea gushed of the No to with of staphylococcic rapid digitalization. progressively time more iliac blood evidence evacuation essentially and sided clot.” hospital. superior of old no that another examination with transfusions. a left in was transferred hospital pursued from there infected was abdominal a Cardiomegaly, with expired patient developed serious week. The the The periphery a anterior ml. blood progression blood material. the therapy, in 1956 Pathologic again tumor initiated. stay but was prevent whole bloody Nevertheless, organism his numerous purulent, antibiotic was during clots, disappeared was The the malignancy right 1000 closed. unhealed, and transfusions old following patient blood. bleeding day controlled in remained ooze control per drain the site 17, the “degenerating the at made October below wound largely over calcification tumor approximately only swelling 1956, biopsy on a few the showed size tissue CHRISTENSEN to his physician with the recommendation and that a needle biopsy obtained. opened, and irregular soft immediately contained original December Hospital. was inserted surgery formation, the attempted made lata abdominal regain was was cavity was of returned with plasma biopsy fascia drain obtained were was incision the Penrose A surgical bone size patient transfused vertical \Vhen new unusual AND of size. The contour. the gross bulky, ilium crest A on The had and lateral lesion was of soft taken place. border of death ( fig. tissue mass The the obviously 2) no of erosion area ilium still demonstrated as had present considerable longer had approached but Definite decreased considerably a a striking improve- apparent. fairly re- normal response to bloody necrotic external therapy had occurred. Autopsy Findings The right iliac measuring ilium. The pus was psoas seen. revealed no fossa evidence polymorphonuclear lobe damage with tis noted and all were necrotic tions stained and which brain. invaded but were the proliferation on microscopic The with Unfortunately, this pleura of the stages an was pulmonary no debilitated, cultures in sarcolemmal hemorrhagic brain tissue heart. and within were from the obtained the were large to the is, exact showed in of of was present small. was present Zones was The There areas a myocardiin vessel present unknown. of the arteritis walls leukocytes. nature the neuronal diffuse The zones therefore, gross evident. was polymorphonuclear inflammatory right No hematonia nuclei. showed There the sections infiltration. and patient of Hemosiderosis fibrotic. and wall area also cell the artery, antibiotic-treated the Numerous increase macrophages mycelia from material in consistency. formation of defect flabby bone large thickened large new A of of taken of with of a macrophages. polymorphonuclear with showed and sections Sections mass was color laden infiltration. infiltrated PAS of atrophic and defined There in examination left branches and brown various brain. glial spleen. involving of a poorly diameter. hemosiderin leukocytic parietal were dark and neoplasm, fibers in examination changes muscle by cm. was Microscopic of adjoining liver occupied 10 muscle fibrotic right was approximately Secin the lung fungus From www.bloodjournal.org by guest on June 17, 2017. For personal use only. PSEUDOTUMOR OF 587 HEMOPHILIA Fic. 2.-Anteroposterior film of pelvis, May 24, 1957. DISCUSSION The first 1918.1:1 because of 8 months were seen into report He the blood patient hemopitilic a a large tumor swelling was These have of the surrounding Little been soft is of hemophilia. The tissue. the process producing bleeding originates regarding exact thigh. large the anatomic anemic evidence of destructive bleeding to bone destruction under the periosteum and this been vell physiology of the are occurred and 750 in hemorrhage uncertain. and produces of The in 10 bones (table 1). swelling pronounced of ml. refilled. expired neoplasm. changes “pseudotumor have pathologic site and aid of the femur with bleeding tendencies1’4’5’6’8’2 hematomas names, had probed 4 days in medical trauma in Starker by sought thinning the bone was sac. became with The Slight swelling tumor fever, with Presented who and periosteal of sarcoma of revealed no extra-articular of bone”1#{176}appear known right of the in 18 patients associated tumor his The collapse developed examination original report, reported “resorption in was hemophiliac destruction the diagnosis entertained. aspirated, with subsequently been Pseudt1mor 14-year-old earlier. Cortical on x-ray and days. Pathologic Since this have of described hemophilia”4 or accepted. of this and complication the It is possible erosion by nature of that the pressure From www.bloodjournal.org by guest on June 17, 2017. For personal use only. 588 SILBER from the outside; increasing hematomas In also patient been noted in dogs taming ceivable originate hematoma in by the through the other Others extension from was under saline At present hemophilia there possesses As has deficiency. has into their continuously account for is the evidence that osteolytic potency the in the development Development in only eight in that months of signs swelling and of therefore, exact of amount preceded cases. The may elapse or even years ) . The customary duration. formation osteitis include fibrosa static normal ulna, tibia and secondary, osteogenic solitary and osteogenic are clearly sarcoma, important may also bleeding tendencies a possibility in even amputation. Needle incision. even are However, this presents continually Therapy, in in PTC plasma deficient. assumption may be Roentgen that presence considerable present. our opinion, it hastens a may aspiration the the underlying coagulation plasma transfusions over or stored develops hemophilia risk. should slowly cell is a safer The dangers aimed at primary clinical festures diagnosis of a patient with of Awareness exploration technic in tumor, meta- both patient biopsy a hematoma may new Differentiation It is apparent mass. the The which and giant cell sarcoma, malignancies, growing save of be affected. considerations. he most difficult. vell appearance lesions elevation the characteristics listed above that all radiologic and osteogenic sarcoma may be duplicated. A presumptive pseudotumor should probably be made in any instance where such injury been painless involved is been an known factors of the from of bleeding is questionable deformans, The sarcoma. the most in particular, of and reticulum with primary history osteolytic periosteal con- due to a PTC in this disorder. the injury osteitis of main- It is contumor of duration are have plasmacytoma, has patients complaint has most frequently thumb This blood.12 was a definite between osteomyelitis, cystica, malignancy and from of of trauma presenting The bone the in the case of or an intramedullary blood and by by that and 12 hours.1’ “resorption met space importance effect necrosis cavities for the defect illium has been the only flat bone involved. The differential diagnosis of such localized show varying degrees of irregular margination, bone pressure. massive than coagulation reported symptoms. The several months 1 the of the of a pseudotumor. this lesion was of the ( table that nature this hemarthroses.7 femoral be no have produced this situation would a confined sub-periosteal greater suspect, than femur old CHRISTENSEN suggested been indicated, our patient’s bleeding tendency This is the first reported instance of pseudotumor would rather of also have considerable Larsen sterile into might shaft. cases.7”2 infusing All requirements minimal oozing cavity. the on a pressure of 180 cm. of water that a similar mechanism may bone.” tinual One hemorrhage pressure may our bone intramedullary internal AND than surgical a hemophiliac patient of hemorrhage and the or correction or infection control of defect. Active bleeding must be stopped. Repeated a long period of time may be required. Fresh plasma given depending therapy resorption has on been of the whether advocated tumor.1#{176} the patient in the is AHG past on or the From www.bloodjournal.org by guest on June 17, 2017. For personal use only. PSEUDOTUMOR OF 589 HEMOPHILIA 1.-Pseudotumor TABLE Number of Age of patients of Bone reported 18 patients 9-55 Sex Affected Bone Femur 10 cases Tibia 3 cases Ilium 3 cases Thumb Fatal 2 cases outcome Surgical to pseudotumor) (related intervention (biopsy, or Duration The mortality 18 because the for of sarcoma) 10 cases 4 months patients reported in In four poor of pseudotumor rate patients complications. to of 7 cases exploration amputation possibility the years Male of the outcome. with the cases, complication died surgical Progression over a period of years mediate outcome is not this literature of and result fatal. is the may untreated serious years high. Seven pseudotumor intervention the in of -24 have its contributed pseudotumor incapacitation of or may even occur if the im- SUMMARY 1. A case of pseudotumor 2. The natural hemophilia of the history are ilium and 1. Es reportate de 2. Es serie of deficient this patient serious is reported. complication of reviewed. SuuIAmuo cientia in a PTC treatment un caso de INTEIILINGUA IN pseudotumor del ilium in un patiente con defi- PTC. presentate un complication revista de del historia natural e del tractamento de iste hemophilia. REFERENCES 1. Becker, F.: Chir. 2. Biggs, Rosemary and platelet Echternacht, count. A. P.: W. M. surgical and A. Hosp. E.: Uber Am. Ni. J. S.: and Clin. Pseudotumor Resorptionsgcschwulst The bei thromboplastin of 59:237, die hemophilic Cronkite, Path. of bone. B.: Woodhall, treatment Hopkins Forfota, Douglas, G., Schneiderman, the 5. Firor, 6. sog. Hiimophilie. generation test. Zentralbi. J. Clin. Path. 1953. 3. Brecher, 4. vortiiuschende 1942. 6:23, of Sarkom 69:113, E.: 23:15, lesions in 14:80, and constancy 1943. pseudotumor: the smaller diagnosis, bones and pathology joints. Bull. and Johns 1936. Gelenk-und Knochenveranderungen 3:399, 1931. 7. Ghormley, R. K. and Clegg, R. S.: Bone and of cases of so-called hemophilic pseudotutnor. 8. G#{252}nsel,E.: Uber krankhafte Veranderungen R#{246}ntgenpraxis Reproducibility 41:565, Radiology Hemophilic The 1953. 1942. joint J. an changes Bone Knochen bei Blutern. in hemophilia, & Joint und Surg. Gelenken R#{246}ntgenpraxis with 30-A:589, bei report 1948. Blutern. From www.bloodjournal.org by guest on June 17, 2017. For personal use only. 590 SILBER 9. Larsen, physeal 10. Muller, R. philie. 11. Petersen, 28:323, 12. 13. 14. M.: Intramedullary bone J. H.: Reinecke necrosis. Uber die A Case Surg. of 1942. osseous changes particular reference CHRISTENSEN to massive din- 1938. von 72:281, in sog. a Resorptionsgeschw#{252}lsten patient with bei hemophilia. Acta H#{228}inoradiol. 1947. and Wohlwill: tYber Hamophilie 1929. Starker, L.: Knochenusur durch Grenzgeb.d.Med. u. Chir. 31:381, Walker, H. J. S. and Vomack, hemophilia. with 108:127, Rontgentherapie Strahlentherapie J.: pressure Ann. AND Arch. Surg. 56:329, Gelenkerkrankung. em Hamophiles, Arch. kIm. subperiostales Chir. H#{228}matom. 154:424, Mitt.a.d. 1918. N. A.: 1948. Pseudotumor of bone in hereditary pseudo- From www.bloodjournal.org by guest on June 17, 2017. For personal use only. 1959 14: 584-590 Pseudotumor of Hemophilia in a Patient with PTC Deficiency R. SILBER and W. R. 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