Literature DB >> 31616120

An Unusual Presentation of Hemophilia A: Pseudotumor of Distal End Radius-Masquerading as Giant Cell Tumor.

Amit Sharma1, Jeetendra Singh Lodhi1, Purushottam Lingaiah1, Awkash Kumar1, Sumit Arora1.   

Abstract

Hemophilia is a coagulation defect caused by a functional or absolute deficiency of coagulation factors. Deficiency of factor VIII leads to hemophilia A; deficiency of factor IX causes hemophilia B. Pseudotumor may arise from hemorrhage into the muscle and within the subperiosteal space and can lead to destruction of the adjacent muscle, bone, nerves, and vessels. A 60-year-old man presented with the complaint of pain and swelling in right wrist. The patient consulted the general practitioner who misdiagnosed it as a case of giant cell tumor due to typical X-ray changes. Biopsy was done following which there was prolonged bleeding leading to worsening of clinical condition and the patient presented in our hospital with ulcerated swelling. Routine blood investigations were within normal limit. Prothrombin time was 12.1 seconds and activated partial thromboplastin time (aPTT) was 54.3 seconds. Raised aPTT led to suspicion of hemophilia, and factor VIII and IX levels were investigated. Factor IX level was within normal limit. Factor VIII level was 6.3%. The patient was managed conservatively. Factor VIII level was replaced according to standard protocol (40 IU/kg per dose thrice a week for 10 days). Daily dressing was done using tranexamic acid locally. Lesion healed completely in 6 months. © Thieme Medical Publishers.

Entities:  

Keywords:  factor IX; factor VIII; hemophilia A; hemorrhage

Year:  2018        PMID: 31616120      PMCID: PMC6791804          DOI: 10.1055/s-0038-1646774

Source DB:  PubMed          Journal:  J Hand Microsurg        ISSN: 0974-3227


  17 in total

Review 1.  The haemophilic pseudotumour.

Authors:  E C Rodriguez-Merchan
Journal:  Haemophilia       Date:  2002-01       Impact factor: 4.287

Review 2.  Haemophilic cysts (pseudotumours).

Authors:  E C Rodriguez-Merchan
Journal:  Haemophilia       Date:  2002-05       Impact factor: 4.287

3.  Pseudotumor occurring in hemophilia.

Authors:  J W van Ommeren; D W Mooren; R P Veth; I R Novakova; C A van de Kaa
Journal:  Arch Orthop Trauma Surg       Date:  2000       Impact factor: 3.067

4.  Hemophilic pseudotumor: radiologic-pathologic correlation.

Authors:  James M Stafford; Tina T James; Anton M Allen; Lisa R Dixon
Journal:  Radiographics       Date:  2003 Jul-Aug       Impact factor: 5.333

5.  Hemophilic pseudotumor of the distal parts of the radius and ulna. A case report.

Authors:  Samir Shaheen; Eltayeb Alasha
Journal:  J Bone Joint Surg Am       Date:  2005-11       Impact factor: 5.284

6.  An unusual presentation of hemophilia B: pseudotumor of proximal tibia.

Authors:  Saurav Mittal; Sumit Arora; Shilpa Khanna; Lalit Maini; V K Gautam
Journal:  Am J Orthop (Belle Mead NJ)       Date:  2011-07

Review 7.  Giant haemophilic pseudotumour of the pelvis: case report and literature review.

Authors:  P H Pennekamp; A C Strauss; C Klein; A Marx; G Goldmann; M Friedrich; N Marquardt; J Oldenburg
Journal:  Haemophilia       Date:  2015-07-14       Impact factor: 4.287

8.  Pelvic haemophilic pseudotumour: a case report.

Authors:  Achmad Fauzi Kamal; Lugyanti Sukrisman; Ismail Hadisoebroto Dilogo; Heka Priyamurti; Muhammad Nurul Qomaruzzaman
Journal:  J Orthop Surg (Hong Kong)       Date:  2014-08       Impact factor: 1.118

9.  Radiologic aspects of hemophilic pseudotumors in bone.

Authors:  E E Brant; H H Jordan
Journal:  Am J Roentgenol Radium Ther Nucl Med       Date:  1972-07

Review 10.  Topical application of tranexamic acid for the reduction of bleeding.

Authors:  Katharine Ker; Deirdre Beecher; Ian Roberts
Journal:  Cochrane Database Syst Rev       Date:  2013-07-23
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