Extramedullary hematopoiesis (EMH) is a rare cause of adrenal mass. We present a 44-year-old woman who has thalassaemia intermedia, referred to Endocrinology clinic for huge adrenal mass. Along with a paraspinal lesion discovered in this patient, the leading diagnosis was EMH. The patient was treated with hypertransfusion and hydroxyurea, which led to a reduction in the size of the right adrenal mass and paraspinal mass. This case highlights the challenges in managing this rare condition. Although EMH is a rare cause of adrenal mass, the diagnosis must be considered in any patient with a history of a congenital hemolytic disorder, to avoid unnecessary surgical procedures.
Hematopoiesis, Extramedullary

Humans ; Hematopoiesis, Extramedullary ; Liver Neoplasms/diagnosis* ; Diagnostic Errors

Transient myeloproliferative disorder (TMD), which may mimic acute leukemia, occurs in neonates with Down syndrome along with hepatic fibrosis. TMD is recognized shortly after birth or in the neonatal period and is characterized by leukocytosis and thrombocytopenia, which resolve spontaneously in four to six weeks. And hepatic fibrosis is characterized by diffuse intralobular sinusoidal fibrosis, extramedullary hematopoiesis and hemochromatosis. A newborn male infant with Down syndrome, atrial septal defect and ventricular septal defect is reported. He showed abnormal myelopoiesis accompanying characteristic hepatic sinusoidal fibrosis. Knowing the cellular mechanism of hepatic fibrosis and its modulation by growth factors, a pathogenetic link between transient myeloproliferative disorder and the development of liver fibrosis in Down syndrome neonates, association of this triad no longer appears to be accidental.
Down Syndrome* ; Fibrosis* ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Hematopoiesis, Extramedullary ; Hemochromatosis* ; Humans ; Infant ; Infant, Newborn ; Intercellular Signaling Peptides and Proteins ; Leukemia ; Leukocytosis ; Liver Cirrhosis ; Male ; Myelopoiesis ; Myeloproliferative Disorders* ; Parturition ; Thrombocytopenia

It has been known that extramedullary hematopoiesis occurring after birth can be developed in various diseases, and it is often found in hematologic diseases. Among these, congenital dyserythropoietic anemia is a rare disease characterized with increase of ineffective hematopoiesis and morphological abnormalities of erythroblasts. In congenital dyserythropoietic anemia, extramedullary hematopoiesis is very rare and only a few cases have been reported. Although treatment is not required if there is no symptom in extramedullary hematopoiesis, surgery or radiation therapy is effective in case that there is symptom or unresponsive anemia despite blood transfusion. This case report is about surgical treatment for extramedullary hematopoiesis in 23-year-old patients diagnosed of congenital dyserythropoietic anemia.
Anemia ; Anemia, Dyserythropoietic, Congenital* ; Blood Transfusion ; Erythroblasts ; Hematologic Diseases ; Hematopoiesis ; Hematopoiesis, Extramedullary* ; Humans ; Parturition ; Rare Diseases ; Young Adult

MRI findings of extramedullary hematopoiesis of the spleen have not been described in the literature. Wereport the MRI features of this condition, as seen in two patients and confirmed by fine needle biopsy. Threesmall masses(< or =3cm) were isointense on T1WI, hyperintense on T2WI, and enhanced after the injection of gadolinium.Two 6cm-sized masses were hypointense on both T1WI and T2WI, and showed no contrast enhancement.
Biopsy, Fine-Needle ; Hematopoiesis, Extramedullary* ; Humans ; Magnetic Resonance Imaging* ; Primary Myelofibrosis* ; Spleen*

A seventy-seven year old female was consulted for radiation therapy due to the infiltration of lung and pleural effusion from myelofibrosis. She was diagnosed clinically as extramedullary hematopoiesis (EMH), and had severe dyspnea and cardiac dysfunction originated from pulmonary hypertension. She underwent low-dose (2 Gy) whole-lung radiation therapy in 4fractions of 50 cGy each. Clinical symptom, especially dyspnea, and chest X-ray imaging was markedly improved after radiation therapy. We experienced that the low-dose whole-lung radiation therapy to EMH in lung might be effective for the palliation of pulmonary hypertension
Dyspnea ; Female ; Hematopoiesis, Extramedullary ; Humans ; Hypertension, Pulmonary ; Lung ; Pleural Effusion ; Primary Myelofibrosis ; Radiotherapy* ; Thorax

Extramedullary hematopoiesis (EMH) is occasionally reported in idiopathic myelofibrosis and is generally found in the liver, spleen, and lymph nodes several years after diagnosis. Myelofibrosis presenting as spinal cord compression, resulting from EMH tissue is very rare. A 39-yr-old man presented with back pain, subjective weakness and numbness in both legs. Sagittal magnetic resonance imaging showed multiple anterior epidural mass extending from L4 to S1 with compression of cauda equina and nerve root. The patient underwent gross total removal of the mass via L4, 5, and S1 laminectomy. Histological analysis showed islands of myelopoietic cells surrounded by fatty tissue, consistent with EMH, and bone marrow biopsy performed after surgery revealed hypercellular marrow and megakaryocytic hyperplasia and focal fibrosis. The final diagnosis was chronic idiopathic myelofibrosis leading to EMH in the lumbar spinal canal. Since there were no abnormal hematological findings except mild myelofibrosis, additional treatment such as radiothepary was not administered postoperatively for fear of radiotoxicity. On 6 month follow- up examination, the patient remained clinically stable without recurrence. This is the first case of chronic idiopathic myelofibrosis due to EMH tissue in the lumbar spinal canal in Korea.
Adult ; *Cauda Equina ; Chronic Disease ; *Hematopoiesis, Extramedullary ; Humans ; Male ; Primary Myelofibrosis/*complications ; Spinal Cord Compression/*etiology

Thoracic extramedullary hematopoiesis (EMH) is a rare disease entity that is usually associated with hematologic disorders, such as myelodysplastic or hemolytic disease. Because thoracic EMH is usually encountered as a mass during radiologic examinations, it should be differentiated from posterior mediastinal neurogenic tumors. Here, the authors report a case of EMH associated with hereditary spherocytosis. The patient underwent a complete excision by thoracoscopic surgery to differentiate it from other mediastinal tumors.
Hematopoiesis, Extramedullary ; Humans ; Mediastinal Neoplasms ; Mediastinum ; Rare Diseases ; Spherocytosis, Hereditary ; Thoracoscopy

Myelofibrosis results from stimulation of bone marrow stromal fibroblasts by fibrogenic cytokines elaborated by neoplastic or reactive cells in the marrow. Chronic idiopathic myelofibrosis should be differentiated from secondary myelofibrosis resulting from bone marrow involvement of malignant lymphoma because these diseases have different therapeutic strategies. Myelofibrosis in systemic lupus erythematosus is an uncommon but well-recognized complication, and identifying an autoimmune myelofibrosis is important in diagnosing this benign cause of myelofibrosis. We report two cases of myelofibrosis presenting the clinical and radiologic findings that mimicked malignant lymphoma -a case of autoimmune myelofibrosis associated with systemic lupus erythematosus showing extensive lymphadenopathy and a case of chronic idiopathic myelofibrosis with focal intrasplenic extramedullary hematopoiesis- and discuss the importance of the clinical information and radiologic findings for the pathologic diagnosis of myelofibrosis.
Abdomen* ; Bone Marrow ; Cytokines ; Diagnosis ; Fibroblasts ; Hematopoiesis, Extramedullary* ; Lupus Erythematosus, Systemic* ; Lymphatic Diseases* ; Lymphoma* ; Primary Myelofibrosis*

Adrenal Glands ; Female ; Hematopoiesis, Extramedullary ; Humans ; Spherocytosis, Hereditary ; complications ; Young Adult