Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia

Tan, Tze-Ching; Tsao, J.I.; Cheung, Fung-Ching

doi:10.1054/jocn.2001.1038

Cited by 50 publications

(50 citation statements)

References 30 publications

(29 reference statements)

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“…In most cases, these masses are incidentally detected on imaging studies of the chest or abdomen [8,9]. In other cases, the masses may produce symptoms compressing neighboring structures [10,11,12,13,14,15,16,17,18,19,20,21,22]. Magnetic resonance imaging (MRI) is the method of choice for diagnosing EMH [7].…”

Section: Introductionmentioning

confidence: 99%

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

et al. 2017

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Extramedullary hematopoiesis (EMH) results from the extension of hematopoietic tissue beyond the confines of the bones. Since the initiation of regular transfusion programs from an early age for all thalassemia major (ΤΜ) patients, EMH has not been considered a clinical issue anymore. The present study aims to record the prevalence of EMH in chronically transfused ΤΜ patients followed at our institution and to investigate possible risk factors associated with its occurrence. The project was designed as a retrospective, nonexperimental, descriptive, exploratory study. In total, the study enrolled 104 patients. EMH was revealed in 15/104 (14%) patients. The presence of intravening sequence (IVS)-I-6 was significantly related with the development of EMH (p < 0.05). No other demographic or biological factor studied was found to be related with the presence of EMH. The study stresses a profound incidence of asymptomatic EMH in a solid group of well-transfused ΤΜ patients. Given the high incidence of the IVS-I-6 allele in the Mediterranean and Middle Eastern region, high-quality, prospective, multicenter studies could confirm the association of EMH occurrence with the presence of the IVS-I-6 mutation and further evaluate the exact role of this mutation in the EMH process.

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Section: Introductionmentioning

confidence: 99%

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

et al. 2017

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“…But this problem can be easily addressed with concomitant steroid therapy. 17 Munn RK in 1988 showed that treatment with steroids and external beam radiation is effective and usually long-lasting. 8 The case reported by us revealed dramatic clinical as well as radiological improvement with radiation therapy in a twenty one year old female thalassemic patient who presented with spinal cord compression due to Extramedullary haematopoiesis.…”

Section: Discussionmentioning

confidence: 99%

Thoracic spinal cord compression due to xtramedullary haemopoiesis in a patient with beta-thalassemia: complete clinical regression with radiation therapy alone

Saha

Chattopadhyay

Azam

et al. 2014

Int J Cancer Ther Oncol

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Case Report AbstractSpinal cord compression due to extramedullary haemopoesis in beta-thalassemia is extremely rare. Controversies are there between the two modalities of treatment surgery vs radiation therapy. We present here a case of beta thalassemia major in a twenty one year old female patient who presented with features of spinal cord compression due to extramedullary haemopoesis. She was then treated with 3000 cGy of radiation therapy targeted to the T5-T8 region, as 200 cGy/fraction daily, 5 fractions/week, over 6 weeks. The patient's haemoglobin was elevated from 6.1g/dl to 10.1g/dl, with her haematocrit rising from 26.3% to 32.8%. Steroid dose was tapered on hospital day number 7. She achieved near full neurological recovery after medical treatment with steroids, blood transfusion and radiation therapy.

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“…Bone marrow insuffi ciency seen in myelofi brosis, carcinomatosis, lymphoma and leukemia can also result in EMH. Rarely can it be seen in pernicious anemia, vitamin B 12 and folate defi ciency [2][3][4][5] . Due to chronic anemia that stimulates erythropoietin production, erythropoiesis is massively increased (10-15 times normal) [6] .…”

Section: Discussionmentioning

confidence: 99%

“…Clinical suspicion may be raised by a history of chronic anemia, such as thalassemia and evidence of EMH elsewhere, such as hepatosplenomegaly or lymphadenopathy [2] .…”

Section: Discussionmentioning

confidence: 99%

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Presacral Extramedullary Hematopoiesis: A Diagnostic Confusion concerning a Rare Presentation

Al-Aabassi

Murad

2005

Med Princ Pract

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Objective: To report a rare case of extramedullary hematopoiesis (EMH) with an asymptomatic intrapelvic mass in a patient with thalassemia major. Clinical Presentation: The patient presented with a pelvic mass discovered by ultrasonography before undertaking laparoscopic cholecystectomy for gallstones. Technetium-99m MDP bone scan failed to differentiate EMH from malignancy. A fine-needle aspiration smear was misdiagnosed when atypical megakaryocytes were interpreted as malignant cells. EMH was suggested by magnetic resonance imaging and confirmed by an ultrasound-guided Tru-Cut biopsy. Conclusion: This case report illustrates the need to consider EMH in the differential diagnosis of patients with chronic hematological disorders and mass lesions in extramedullary sites.

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Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia

Cited by 50 publications

References 30 publications

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

Presence of the IVS-I-6-Mutated Allele in Beta-Thalassemia Major Patients Correlates with Extramedullary Hematopoiesis Incidence

Thoracic spinal cord compression due to xtramedullary haemopoiesis in a patient with beta-thalassemia: complete clinical regression with radiation therapy alone

Presacral Extramedullary Hematopoiesis: A Diagnostic Confusion concerning a Rare Presentation

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