Extramedullary hematopoiesis presenting as intrathoracic tumors.Report of a case in a patient with thalassemia minor

Knoblich, Roman

doi:10.1002/1097-0142(196005/06)13:3<462::aid-cncr2820130308>3.0.co;2-7

Cited by 54 publications

(12 citation statements)

References 10 publications

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“…In the past the diagnosis of intrathoracic ex- tramedullary haematopoietic masses of thalassaemic patients was suspected from the typical osseous abnormalities found in chest radiographs [2,4]. Diagnosis was established with biopsy of the mass after its surgical removal [1,7].Diagnosis was later established by computed tomography, which has now been replaced by MRI [11]. MRI of the spinal cord with its characteristic findings is now considered the most appropriate investigation of thalassaemic patients who present with neurological symptoms due to spinal cord compression.…”

Section: Discussionmentioning

confidence: 99%

“…Extramedullary haematopoiesis is a common manifestation in a variety of haematological disorders such as severe anaemia, erythraemia, leukaemia, hereditary haemoglobinopathies, osteosclerosis, carcinomatosis, Hodgkin' s disease, conditions following haemorrhage [1], hereditary spherocytosis, erythroblastosis foetalis and myelofibrosis [2]. In the above conditions production of blood elements by bone marrow is insufficient for the demands of the circulation.…”

Section: Introductionmentioning

confidence: 99%

“…In the above conditions production of blood elements by bone marrow is insufficient for the demands of the circulation. Because of this organs such as liver, spleen and lymph nodes and rarely other tissues such as thymus, adrenal cortex, lungs and pleura, appendix, dura mater, breast, sciatic nerve, retroperitoneum and paravertebral area of the thorax [1] become sites of extramedullary haematopoiesis.…”

Section: Introductionmentioning

confidence: 99%

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Spinal cord compression due to extramedullary haematopoiesis in two patients with thalassaemia: complete regression with blood transfusion therapy

Aliberti

Patrikiou

Terentiou

et al. 2001

Journal of Neurology

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Extramedullary haematopoiesis, a common finding in thalassaemia, is rarely localized in the spinal cord and even more rarely has neurological manifestations. We present two patients suffering from thalassaemia (intermedia and beta homozygous) and paraparesis due to spinal cord compression from intrathoracic extramedullary haematopoietic masses. Diagnosis was based on magnetic resonance imaging findings, and treatment consisted of blood hypertransfusions. The majority of published cases have been successfully treated by radiation and in some cases by blood transfusions. Our two patients completely recovered, and there has been no recurrence during the 4 years since their treatment. Diagnosis and treatment of this rare condition are discussed.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Spinal cord compression due to extramedullary haematopoiesis in two patients with thalassaemia: complete regression with blood transfusion therapy

Aliberti

Patrikiou

Terentiou

et al. 2001

Journal of Neurology

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“…It occurs due to expansion of the haemopoietic tissue in order to supplement marrow production. Knoblich [3] proposed that multipotential cells in the epidural space can be transformed into marrow under conducive circumstances, and Lyall [4] advocated the possibility of direct extension from marrow into the epidural space. Occasionally it appears as a tumor-like mass causing compression of spinal cord [5,10,11,12,13].…”

Section: Discussionmentioning

confidence: 99%

MRI findings of extramedullary haemopoiesis

et al. 2001

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Extramedullary haemopoiesis (EH) is a compensatory process associated with chronic haemolytic anaemia. It is rare, however, for such an abnormality to cause spinal cord compression. We present two patients with known beta-thalassaemia intermedia who developed spinal cord compression due to masses of extramedullary haematopoietic tissue in the epidural space of the thoracic spine. The EH masses were diagnosed by MRI as an isointense epidural lesion on both T1- and T2-weighted images, compressing severely the spinal cord. After administration of a paramagnetic agent, an intermediate enhancement of the masses was evident. All the vertebral bodies had low to intermediate signal intensity as a result of displacement of fatty marrow by haematopoietic marrow. Expansion of thoracic ribs with bilateral paravertebral masses were characteristic. A small dose of radiotherapy was given and marked improvement in neurological symptoms was evident. An MRI examination established shrinkage of the mass and decompression of spinal cord. The role of MRI in diagnosis of EH masses is essential and radiation therapy is a very effective treatment for this rare complication.

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“…His patient suffered from acholuric jaundice. KNOBLICH (1960) reported a case of ectopic hemopoiesis in the thorax, found after surgical exploration in a patient in whom the process was detected roentgenologically but was misinterpreted as a neurofibroma. The patient had Cooley's anemia which was not diagnosed until the nature of the thoracic tumours was known.…”

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confidence: 99%