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At least 19 recordsLinked to original sources
PubMed2006

Surgical treatment of an unusual case of pelvic extramedullary hematopoiesis.

Extramedullary hematopoiesis affects about 15% of the patients treated for thalassemia intermedia. Usually seen in adulthood, the most common location is the paraspinal region. Diagnosis and treatment of extramedullary hematopoiesis located in the pelvis of a young 15-year-old girl is discussed. The young age of the patient and the uncommon site of the mass first lead to the diagnosis of an ovarian dermoid cyst. Because of the clinical history and the typical feature on computed tomography scan, extramedullary hematopoiesis was concluded. A specific treatment based on blood transfusion and hydroxyurea was first proposed but remained inefficient. Surgical excision was thus successfully performed. Whereas surgery is limited to spinal cord compression in paraspinal extramedullary hematopoiesis, this observation argues for surgical treatment in symptomatic intraabdominal extramedullary hematopoiesis when medical treatment fails.

Adolescent↗
PubMed2004

Intrathoracic masses due to extramedullary hematopoiesis.

Extramedullary hematopoiesis often occurs in hemoglobinopathies, hemolytic anemias, leukemias, lymphomas, and myeloproliferative disorders. Liver, spleen, and lymph nodes are frequently involved. However, extramedullary hematopoiesis may also develop in other sites such as thymus, kidney, retroperitoneum, and paravertebral areas of the thorax. Extramedullary hematopoietic masses are often microscopic and asymptomatic, but sometimes they lead to tumor-like masses. We describe massive intrathoracic extramedullary hematopoiesis in a 41-year-old man with compound heterozygosis for beta-thalassemia and sickle cell anemia and functional asplenia. We also describe a 39-year-old man with beta-thalassemia intermedia, who was initially diagnosed as having tumor masses, but was later proved, by magnetic resonance imaging, to have extramedullary erythropoietic tissue. These observations provide further support to include extramedullary hematopoiesis among the differential diagnosis of tumor-like masses in patients with hematologic diseases.

Adult↗
PubMed1992

[The diagnostic imaging of intrathoracic extramedullary hematopoiesis].

Extramedullary hematopoiesis is a rare finding. It represents a compensatory mechanism capable of assuring and keeping sufficient erythrogenesis. The distribution of the main locations of extramedullary hematopoiesis was observed in different organs and apparatuses. According to their experience, the authors investigated the potentials and limitations of conventional radiology, of CT and MR Imaging, to diagnose intrathoracic extramedullary hematopoiesis in the posterior mediastinum. While stressing the value of clinical and anamnestic data and of conventional radiology, the authors emphasize the leading role of CT and MR imaging. As a matter of fact, the latter is the method of choice, thanks to higher contrast resolution and to its multiplanar and multiparameter capabilities, in the study of space-occupying lesions in the costo-vertebral region. On the other hand, MR Imaging fails to detect calcic areas in paravertebral masses; moreover, the exhaustive and morphostructural analysis of bone segments, ribs and vertebrae, is allowed only by CT.

Adolescent↗
PubMed1987

Ultrasound appearance of extramedullary hematopoiesis.

Extramedullary hematopoiesis accompanies a wide variety of diseases and is defined as the abnormal production of blood outside the normal confines of the bone marrow. Most of the time, extramedullary hematopoiesis is found as microscopic foci in soft tissues; however, on occasion this process may reach a size that is detectable with ultrasound. Two forms are possible: "paraosseous"--in which the normal medullary tissue of the bone marrow ruptures through the bone to present as a paraosseous soft tissue mass,--and "extraosseous"--in which extramedullary hematopoiesis occurs within soft tissue. These "masses" of hematopoietic tissue can appear almost anywhere in the abdomen and pelvis and can mimic a wide variety of more serious diseases.

Adolescent↗
PubMed2001

Paratracheal extramedullary hematopoiesis.

Extramedullary hematopoiesis (EMH) is a rare finding in hematology. A 73-year-old female patient with a 1-week history of severe progressive dyspnea was examined, and computed tomography (CT) showed a paratracheal mass 3 cm in size located 1 cm below the vocals cords and causing obliteration of the tracheal airway. Cytology of a needle biopsy revealed EMH. External radiotherapy of 200-cGy fractions to a total dose of 2000 cGy was administered with 3-dimensional conformal planning to treat the progressive symptoms. The patient's clinical symptoms started to improve 2 days after radiotherapy and had completely disappeared after 7 days. CT scans showed complete response on follow-up at 1 week to 5 months after radiotherapy. Mature and immature hematopoietic cells and many adipose cells were seen in the pretreatment samples. Histologic findings in the posttreatment samples showed that these cells had completely disappeared due to the conformal radiotherapy. On the basis of clinical, radiologic, and histologic results, we suggest that conformal radiotherapy may be useful for the treatment of paratracheal localization of EMH because good tumoral irradiation was obtained in this case, with the protection of normal tissues.

Aged↗
PubMed1995

Acute respiratory failure due to extramedullary hematopoiesis.

Extramedullary hematopoiesis (EMH) associated with myelofibrosis uncommonly occurs within the thorax. We describe the first reported case of acute and rapidly fatal respiratory failure due to pulmonary interstitial EMH associated with myelofibrosis. Interstitial EMH should be considered in the differential diagnosis of patients with interstitial pulmonary infiltrates and respiratory failure accompanying a disease process known to predispose to the development of EMH.

Acute Disease↗
PubMed1984

Pelvic extramedullary hematopoiesis.

Extramedullary hematopoiesis is an infrequent cause of thoracic masses. Extrathoracic locations are even less common. We have studied two patients, one with suspected pelvic neoplasm, with technetium-99m sulfur colloid marrow image and transmission computerized tomography. This noninvasive evaluation established the diagnosis of pelvic extramedullary hematopoiesis in both. We recommend a similar diagnostic approach in patients with chronic anemia or other predisposing disease, presenting with a pelvic or abdominal mass.

Adult↗
PubMed1999

Ascites and pleural effusion secondary to extramedullary hematopoiesis.

Extramedullary hematopoiesis in the pleura and peritoneum is rare. It is usually asymptomatic and generally is diagnosed on post mortem examination. Herein we describe a 33-year-old woman with long-standing myelofibrosis who presented with symptomatic ascites and pleural effusion. After complete evaluation, these were found to have been caused by extramedullary hematopoietic implants to the pleura and peritoneum. The pleural effusion responded to low-dose radiotherapy.

Adult↗
PubMedDate not supplied

MR imaging of intrathoracic extramedullary hematopoiesis.

Intrathoracic extramedullary hematopoiesis is a rare neoplasm that is most often asymptomatic and usually discovered during a screening examination for another problem. We present a case of intrathoracic extramedullary hematopoiesis in a patient with homozygous sickle cell disease with CT and magnetic resonance correlation.

Adult↗
PubMed1991

Extramedullary hematopoiesis in cerebellar hemangioblastoma.

Extramedullary hematopoiesis and paraneoplastic erythrocytosis are uncommon in cerebellar hemangioblastoma, and their concomitant occurrence has not been reported. In a study of 26 cases of hemangioblastoma, we found extramedullary hematopoiesis in 4 tumors; one was associated with erythrocytosis. The significance of this association and the possible histogenetic origin of extramedullary hematopoiesis in cerebellar hemangioblastoma are discussed.

Adolescent↗
PubMed2002

[Extramedullary hematopoiesis in the paravertebral space].

BACKGROUND: Extramedullary hematopoiesis is a response of the organism to a deficient production of blood-forming cells within the bone marrow. It may coincide with some hematologic diseases. Two patients with paravertebral mass lesions representing extramedullary hematopoiesis are discussed. Characteristic findings of extramedullary hematopoiesis are presented along with a review of the literature on this topic. CASE REPORTS: A 76-year-old male with a known myelodysplastic syndrome presented with pneumonia. In addition, he had symptoms of a cauda equina syndrome with complaints of pain and hypesthesia of the lower limbs as well as urinary retention. A 63-year-old female presented with aggravated complaints of the lower thorax, low back pain radiating to the upper left leg, and dysesthesia of both feet. In her past medical history, she had polycythemia vera and a splenectomy. Both patients showed paravertebral and intraspinal lesions located in the thoracic and sacral spine which were regarded as extramedullary hematopoiesis according to the imaging findings in MRI and CT. Radiation therapy showed marked improvement in their neurologic complaints following the initial sessions. CONCLUSION: Clinical presentation, knowledge of the underlying disease and of imaging findings are essential in the diagnosis of extramedullary hematopoiesis. MRI is the imaging modality of choice in the primary diagnosis of extramedullary hematopoiesis. Possible extension of the disease into the intraspinal space can be evaluated with high accuracy and differential diagnosis can be facilitated. In addition, MRI is of use in the accurate planning of radiation fields as well as during follow-up of extramedullary hematopoiesis.

Aged↗
PubMed1991

Presacral extramedullary hematopoiesis in thalassemia intermedia.

Extramedullary hematopoiesis is manifested by several clinical hematologic disorders including beta-thalassemia major and thalassemia intermedia. We report an unusual case of extramedullary hematopoiesis, which presented as a painful presacral mass in a patient with beta-thalassemia intermedia. The diagnosis was confirmed by a needle biopsy guided by computed tomography. The patient responded well to radiation therapy to the sacral region.

Adult↗
PubMed1998

Spinal cord compression secondary to extramedullary hematopoiesis in thalassemia intermedia.

Extramedullary hematopoiesis associated with thalassemia causing spinal cord compression is an extremely rare event in the course of the disease. Documentation with an imaging technique, such as MRI, is mandatory. A patient with thalassemia intermedia, who developed paraparesis in spite of transfusion, underwent surgical decompression. Rapid neurological improvement was observed postoperatively and this neurological condition was protected with adequate hemoglobin level. Management of these patients remains controversial. Various modes of therapy such as surgical decompression, radiotherapy, and transfusion are discussed and the related literature is reviewed.

Adult↗
PubMed2000

Myocardial extramedullary hematopoiesis: a clinicopathologic study.

Extramedullary hematopoiesis (EMH) after fetal development is uncommon and is most often seen in patients who have hematologic disorders. EMH unassociated with hematologic disease is rare. After the recent observation of EMH in a myocardial infarct, we sought to determine the frequency and clinicopathologic setting of EMH in myocardial tissues submitted for pathologic examination. Hematoxylin and eosin (H&E)-stained sections from 805 consecutive myocardial samples (207 surgical specimens, 598 autopsy specimens) were examined retrospectively. The presence of immature erythroid or myeloid cell clusters in intramyocardial capillaries or stroma was considered sufficient for the diagnosis of EMH. Immunoperoxidase studies confirming the nature of the hematopoietic cell infiltrate were performed in selected cases. Foci of EMH (often multiple) were identified in 15 of 207 surgical hearts (7.2%) and in 22 of 598 autopsy hearts (3.7%). Patient ages (exclusive of premature infants) ranged from 2 weeks to 73 years (median, 13 years). Twenty-four of 37 (65%) EMH-positive cases were associated with infarcts in various stages of repair (accounting for 11 of 68 [16.2%] of all infarcts in surgical specimens and 13 of 86 [15.1%] of infarcts in autopsy specimens). Acute infarcts less than 72 hours old, excluding those with acute extension, were not associated with EMH. Viral myocarditis and myocardial hypertrophy with fibrosis accounted for primary diagnoses in the nonischemic, EMH-positive surgical cases, whereas seven of nine nonischemic, EMH-positive autopsy cases involved premature or term infants with no obvious myocardial disease. Another autopsy patient had sarcoidosis with myelophthisic involvement of her bone marrow and represented one of only two cases overall in which a hematopoietic disorder was coexistent or suspected. Myocardial EMH is relatively common after myocardial infarct but is rarely encountered in normal or nonischemic myocardium. Its presence in healing but not early acute stages of infarct suggests that EMH results from inflammation- or repair-associated trophic factors, not from ischemia itself.

Adolescent↗
PubMed2002

Extramedullary hematopoiesis in the endometrium.

Extramedullary hematopoiesis (EMH) in the endometrium is an extremely rare occurrence. Four of the eight previously reported cases were related to an underlying hematological disorder, although the remainder had no such relationship. We describe a case of endometrial EMH associated with retained products of conception after termination of pregnancy. Routinely and immunohistochemically stained slides revealed several collections of normoblasts and granulocytic precursors in the endometrium with synchronous chronic endometritis. Retained chorionic villi were also identified. The patient had no known history of a hematological disorder or systemic disease and no such abnormality was detected after detailed hematological work-up. Local effects of growth factors on circulating stem cells may play a pathogenetic role in this process, although an association with recent pregnancy in this case suggests that implantation of fetal hematopoietic elements from the fetus or yolk sac may be more likely.

Abortion, Induced↗
PubMed1999

Idiopathic myelofibrosis with extramedullary hematopoiesis in the kidneys.

Extramedullary hematopoiesis is a common finding in idiopathic myelofibrosis and is usually found in liver and spleen. We report on a patient with biopsy-proven myeloid metaplasia and fibrosis of the renal parenchyma as a rare cause of chronic renal failure. The renal biopsy specimen showed numerous infiltrates of hematopoietic cells expressing growth factors like M-CSF, GM-CSF, IL-1beta and PDGF while TGF-beta was not elevated. These findings suggest that hematopoietic growth factors play a key role in the pathogenesis of this condition causing proliferating fibrosis and enlargement of the kidneys.

Aged↗