Limits...
Extramedullary hematopoiesis in adrenal gland. An uncommon cause of adrenal incidentaloma in sickle cell disease.

Azarpira N, Heidari Esfahani M, Paydar S - Iran J Pediatr (2014)

View Article: PubMed Central - PubMed

Affiliation: Transplant Research Center, ; Department of Pathology.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Extramedullary hematopiesis (EMH) is a response to insufficient blood cell production by producing of blood elements outside of the marrow cavity... Here we report a case of adrenal gland EMH as adrenal incidentaloma in a patient with sickle cell anemia; in literature review no similar result is found... EMH is a rare cause of incidentaloma and is seen in patients with hematologic disorders such as beta-thalasemia, agnogenic myeloid metaplasia and hereditary spherocytosis... EMH is seen in hemoglobinopathies such as thalassemia, hereditary spherocytosis and in hematologic diseases including myelofibrosis and myeloproliferative disorders... In the literature, most cases were associated with intermediate thalassemia... The frequency of extramedullary hematopiesis in thalassemia major was very low, especially, when ineffective erythropoiesis was suppressed by regular transfusion... EMH is rare in patients with sickle cell disease... EMH is usually asymptomatic and discovered incidentally... Symptomatic cases occur due to mass effect with compression to adjacent organ... Treatment options for patients with EMH are described for thalassemia patients and depend on the location and symptoms... Hydroxyurea stimulates the synthesis of hemoglobin F and therefore participates in inactivation and shrinking of EMH... This effect was documented in thalassemia diseases... In conclusion, extramedullary hematopiesis should be considered in the differential diagnosis of patients with sickle cell disease that present with a solitary mass... The CT-guided biopsy can exclude a true neoplasm and alter the management and prognosis.

No MeSH data available.


Right supra renal mass.
© Copyright Policy
Related In: Results  -  Collection


getmorefigures.php?uid=PMC4442845&req=5

Figure 1: Right supra renal mass.

Mentions: Laboratory investigation showed hemoglobin 11 g/dL, WBC count 1.0×109/ L, platelet count 390×109/ L, MCV 70.1 fl, MCH 21.6 pg. Biochemical investigations were: serum bilirubin 1.8 mg/dL; conjugated bilirubin 0.8 mg/ dL, blood urea 27 mg/ dL, serum creatinine 0.7 mg/dL, serum calcium 8.2 mg/ dL, serum phosphorous 6.6 mg/dL, fasting blood sugar 100 mg/dL; alanine aminotransferase 41 IU/ dL, aspartate aminotransferase 50 IU/dL. Tests for hepatitis C virus (anti HCV antibody) and HIV antibodies (Anti HIV-1,2) serum HBsAg and HBc antibody were negative. Fasting serum cortisol level was 13 mcg/dL. Urinalysis was normal. Abdominal sonography showed mild hepatomegaly and a well-defined left suprarenal solid mass in size of 7.7×5.3 cm. Abdominal CT scan confirmed a 7cm well defined suprarenal mass (Fig 1).


Extramedullary hematopoiesis in adrenal gland. An uncommon cause of adrenal incidentaloma in sickle cell disease.

Azarpira N, Heidari Esfahani M, Paydar S - Iran J Pediatr (2014)

Right supra renal mass.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4442845&req=5

Figure 1: Right supra renal mass.
Mentions: Laboratory investigation showed hemoglobin 11 g/dL, WBC count 1.0×109/ L, platelet count 390×109/ L, MCV 70.1 fl, MCH 21.6 pg. Biochemical investigations were: serum bilirubin 1.8 mg/dL; conjugated bilirubin 0.8 mg/ dL, blood urea 27 mg/ dL, serum creatinine 0.7 mg/dL, serum calcium 8.2 mg/ dL, serum phosphorous 6.6 mg/dL, fasting blood sugar 100 mg/dL; alanine aminotransferase 41 IU/ dL, aspartate aminotransferase 50 IU/dL. Tests for hepatitis C virus (anti HCV antibody) and HIV antibodies (Anti HIV-1,2) serum HBsAg and HBc antibody were negative. Fasting serum cortisol level was 13 mcg/dL. Urinalysis was normal. Abdominal sonography showed mild hepatomegaly and a well-defined left suprarenal solid mass in size of 7.7×5.3 cm. Abdominal CT scan confirmed a 7cm well defined suprarenal mass (Fig 1).

View Article: PubMed Central - PubMed

Affiliation: Transplant Research Center, ; Department of Pathology.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Extramedullary hematopiesis (EMH) is a response to insufficient blood cell production by producing of blood elements outside of the marrow cavity... Here we report a case of adrenal gland EMH as adrenal incidentaloma in a patient with sickle cell anemia; in literature review no similar result is found... EMH is a rare cause of incidentaloma and is seen in patients with hematologic disorders such as beta-thalasemia, agnogenic myeloid metaplasia and hereditary spherocytosis... EMH is seen in hemoglobinopathies such as thalassemia, hereditary spherocytosis and in hematologic diseases including myelofibrosis and myeloproliferative disorders... In the literature, most cases were associated with intermediate thalassemia... The frequency of extramedullary hematopiesis in thalassemia major was very low, especially, when ineffective erythropoiesis was suppressed by regular transfusion... EMH is rare in patients with sickle cell disease... EMH is usually asymptomatic and discovered incidentally... Symptomatic cases occur due to mass effect with compression to adjacent organ... Treatment options for patients with EMH are described for thalassemia patients and depend on the location and symptoms... Hydroxyurea stimulates the synthesis of hemoglobin F and therefore participates in inactivation and shrinking of EMH... This effect was documented in thalassemia diseases... In conclusion, extramedullary hematopiesis should be considered in the differential diagnosis of patients with sickle cell disease that present with a solitary mass... The CT-guided biopsy can exclude a true neoplasm and alter the management and prognosis.

No MeSH data available.