Email this article Pseudotumor of the maxilla as first presentation of hemophilia B in a 1-year-old male
- Authors: Kalinina M.P.1, Pshonkin A.V.1, Grachev N.S.1, Poletaev A.V.1, Fedorova D.V.1, Zharkov P.A.1
-
Affiliations:
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
- Issue: Vol 20, No 1 (2021)
- Pages: 156-159
- Section: CLINICAL OBSERVATIONS
- Submitted: 20.04.2021
- Accepted: 20.04.2021
- Published: 20.04.2021
- URL: https://hemoncim.com/jour/article/view/491
- DOI: https://doi.org/10.24287/1726-1708-2021-20-1-156-159
- ID: 491
Cite item
Full Text
Abstract
Pseudotumor is a rare complication of hemophilia with a prevalence 1–2% of all clinical cases. Commonly bone lesions affect long tubular bones, the pelvis, wrist and foot bones. According to world science literature only 16 cases of maxilla bone lesions in patients with hemophilia were found. This article describes a clinical case of maxilla pathological lesion with the destruction of facial skeleton bones in a one year old boy. The differential diagnosis included malignant and benign neoplasia, inflammatory processes. During laboratory examination a deficiency of IX coagulation factor was revealed and the patient was diagnosed with hemophilia В. The patient underwent surgical treatment by removing the lesion with specific replacement therapy; an intraosseous organizing hematoma was verified. The maxilla pseadotumor in this child was a manifestation of hemophilia. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.
Keywords
About the authors
M. P. Kalinina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
Author for correspondence.
Email: lormp@yandex.ru
ORCID iD: 0000-0002-6839-3918
an otorhinolaryngologist at the Department of Oncology and Pediatric Surgery,
1 Samory Mashela St., Moscow 117997
Russian FederationA. V. Pshonkin
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-2057-2036
Moscow
Russian FederationN. S. Grachev
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-4451-3233
Moscow
Russian FederationA. V. Poletaev
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0001-5209-2099
Moscow
Russian FederationD. V. Fedorova
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0003-4567-1871
Moscow
Russian FederationP. A. Zharkov
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0003-4384-6754
Москва
Russian FederationReferences
- Starker L. Knochenusur durch ein hamophiles, subperiostales Hamatom. Mitt Grenzgeb Med Chir 1918; 31: 381– 415.
- Ahlberg A.K. On the natural history of hemophilic pseudotumor. J Bone Joint Surg Am 1975; 57 (8): 1133–6.
- Gilbert M.S. Characterizing the hemophilic pseudotumor. Ann NY Acad Sci 1975; 240: 311–5. 4. Rodriguez-Merchan E.C. The haemophilic pseudotumour. Haemophilia 2002; 8 (1): 12–6.
- Correra A., Buckley J., Roser S., Schreiber A., Syrop S. Radiotherapy of a pseudotumor in a hemophiliac with Factor VIII inhibitor. Am J Pediatr Hematol Oncol 1984; 6 (3): 325–7.
- Cox D.P., Solar A., Huang J., Chigurupati R. Pseudotumor of the mandible as first presentation of hemophilia in a 2-yearold male: a casе report and review of jaw pseudotumors of haemophilia. Head and Neck Pathol 2011; 5: 226–32.
- Rodriguez-Merchan E.C., Gomez-Car dero P. Pathological fracture of a true tumour mimicking a haemophilic pseudotumor. Haemophilia 2005; 11 (2): 188–90.
Supplementary files
