ABSTRACT
Hemophilic pseudotumor is an uncommon complication seen in approximately 1-2% of patients with severe hemophilia. Hemophilic pseudotumors are distinguished into two subdivisions based on location, proximal or distal. Plain x-rays and CT are useful in diagnosis, but MR imaging is the diagnostic test of choice because of its sensitivity to the various blood products. The choice of therapy depends on many parameters, such as the size of the tumor, the age of the patient, and the relation with underlying organs. In most cases of asymptomatic hemophilic pseudotumor, conservative treatment with administration of missing factor as well as immobilization is recommended. The authors describe a 13-year-old boy with severe hemophilia A, who presented with a tibial pseudotumor a few months after an injury. He was conservatively treated for a long period, with daily administration of recombinant factor VIII. His clinical condition improved shortly after therapy induction, but radiological improvement has been moderate. Case history, imaging findings, and therapeutic options are discussed.
Subject(s)
Bone Neoplasms/diagnostic imaging , Hemophilia A/diagnostic imaging , Tibia/diagnostic imaging , Adolescent , Bone Neoplasms/drug therapy , Bone Neoplasms/etiology , Diagnosis, Differential , Factor VIII/administration & dosage , Hemophilia A/complications , Hemophilia A/drug therapy , Humans , Magnetic Resonance Imaging , Male , Time Factors , Tomography, X-Ray ComputedSubject(s)
Respiratory Distress Syndrome/etiology , Transfusion Reaction , beta-Thalassemia/therapy , Adolescent , Female , HumansSubject(s)
Hypoparathyroidism/etiology , beta-Thalassemia/complications , Adolescent , Adult , Child , Child, Preschool , Female , Humans , MaleABSTRACT
Hemoglobinopathies are very common in Greece, the incidence of beta-thalassemia trait being 8% and that of sickle cell trait ranging from 1 to 32% in various districts. In Greek populations, sickle cell disease (SCD) is mainly represented by S-beta thalassemia.
Subject(s)
Anemia, Sickle Cell/complications , Osteonecrosis/surgery , Adolescent , Child , Greece , Humans , Male , Osteonecrosis/etiology , Osteonecrosis/therapy , Osteotomy , Treatment OutcomeABSTRACT
The term "absolute erythrocytosis" denotes a heterogeneous group of disorders characterized by an increased red blood cell mass. The authors describe a 20-month-old girl with absolute erythrocytosis. Erythropoietin levels were found to be extremely increased, although extensive evaluation failed to reveal a cause for such an inappropriate increase. Of interest is also the documentation of spontaneous erythroid colony formation in the patient's bone marrow cultures. Although such a finding is considered typical of polycythemia vera, the diagnostic criteria of this myeloproliferative disorder were not met.