Extramedullary Hematopoiesis-Related Pleural Effusion: The Case of β-Thalassemia

Aessopos, Athanasios; Tassiopoulos, Stergios; Farmakis, Dimitrios; Moyssakis, Ioannis; Kati, Maria; Polonifi, Katerina; Tsironi, Maria

doi:10.1016/j.athoracsur.2006.01.026

Cited by 27 publications

(22 citation statements)

References 27 publications

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“…1, we hypothesize that the increased amount of sTfR associated with splenectomy may have caused a drop in test sensitivity and specificity (from 100% to 78.3% and from 90.9% to 72.2%, respectively). Further studies are needed to confirm and refine this relation including in the radiologic scan also the evaluation of EMH arising from all other districts of the body [10][11][12][13]. However, we found also in our series that the level of sTfR strongly correlated with the need of chelation therapy.…”

Section: Discussionsupporting

confidence: 43%

A useful relationship between the presence of extramedullary erythropoeisis and the level of the soluble form of the transferrin receptor in a large cohort of adult patients with thalassemia intermedia: a prospective study

et al. 2011

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In thalassemia intermedia (TI), the increase in bone marrow hemopoietic activity frequently leads to extramedullary erythropoeisis (EMH), but its relationship with the soluble form of transferrin receptor (sTfR) which fully reflects the marrow erythropoietic activity, has not yet been explored. From January 2007 to December 2010, all TI patients attending at our center were prospectively enrolled to undergo sTfR assay and MRI or CT (if claustrophobic) scan evaluation for the presence of paraspinal EMH. A total of 59 patients with TI were studied; EMH involved 23 (39%) patients; overall, the concentration of sTfR varied from 2.6 to 20.6 (mean = 8.7) mg/L, but in splenectomized group and in unsplenectomized group, it varied from 4.2 to 17.8 (mean ± SD = 9.86 ± 3.33) mg/L and from 2.6 to 20.6 (mean ± SD = 7.25 ± 3.9) mg/L, respectively with a statistically significant intergroup difference (p < 0.01). The cutoff point at 8.6 mg/L using the ROC curve showed a sensitivity of 78.3% and a specificity of 72.2%, in predicting EMH but, in unsplenectomized subgroup, they raised to 100% and 90.9%, respectively. These data showed that in TI the level of sTfR could represent a predictive factor of EMH particularly in patients with spleen.

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Section: Discussionsupporting

confidence: 43%

A useful relationship between the presence of extramedullary erythropoeisis and the level of the soluble form of the transferrin receptor in a large cohort of adult patients with thalassemia intermedia: a prospective study

et al. 2011

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“…Immunohistochemistry results suggested the existence of T lymphocytes (CD3, CD43), B lymphocytes (CD20, CD79a), and hematopoietic elements (MPO, CD15) that were consistent with changes in EMH. Due to death during CT-guided puncture in another report [2], we performed the biopsy under direct vision to minimize bleeding.…”

Section: Discussionmentioning

confidence: 99%

“…These patients often had no respiratory symptoms, and diagnosis was made at autopsy. However, several patients presented with hemothorax or chylothorax [2, 4, 5]. Mechanisms underlying pleural effusion remain unknown.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Pleural Effusion in a Patient with an Extensive Extramedullary Hematopoietic Mass

Luo

Zhang

Lou

2013

Case Reports in Hematology

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We present a 56-year-old woman with bilateral pleural effusions, widespread enlarged lymph nodes, and soft tissue masses located within the renal pelvis. The initially working diagnosis was tuberculosis and lymphoma. Further pathological examination of the lymph node biopsy confirmed a diagnosis of extramedullary hematopoiesis, and a bone marrow biopsy revealed myelofibrosis. Unlike common treatment options such as radiotherapy and/or surgery, intrathoracic cisplatin and dexamethasone for the treatment of pleural effusions secondary to extramedullary hematopoiesis demonstrated an improvement in feasibility and efficacy in the present case.

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“…The common ones are spleen, liver, lymph nodes, and thymus. Rarely, EMH has been reported in the cardiac tissue, breasts, renal tissue, adrenal glands, pleura, retroperitoneal tissue, skin, prostate, broad ligaments, peripheral and cranial nerves, and the spinal canal [3–5]. Reports indicate that the dura mater may have hematopoietic capacity during fetal life which becomes re-activated in pathologic states [6].…”

Section: Introductionmentioning

confidence: 99%

Extramedullary hematopoiesis presented as cytopenia and massive paraspinal masses leading to cord compression in a patient with hereditary persistence of fetal hemoglobin

et al. 2016

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Background: Extramedullary hematopoeisis (EMH) can occur in various physiological and pathologic states. The spleen is the most common site of EMH. Case presentation: We report a case with hereditary persistence of fetal hemoglobin with extramedullary hematopoiesis presented as cord compression and cytopenia secondary to multi-paraspinal masses. Conclusion: Treatment can be a challenge. Relapse is a possibility.

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Extramedullary Hematopoiesis-Related Pleural Effusion: The Case of β-Thalassemia

Cited by 27 publications

References 27 publications

A useful relationship between the presence of extramedullary erythropoeisis and the level of the soluble form of the transferrin receptor in a large cohort of adult patients with thalassemia intermedia: a prospective study

A useful relationship between the presence of extramedullary erythropoeisis and the level of the soluble form of the transferrin receptor in a large cohort of adult patients with thalassemia intermedia: a prospective study

Bilateral Pleural Effusion in a Patient with an Extensive Extramedullary Hematopoietic Mass

Extramedullary hematopoiesis presented as cytopenia and massive paraspinal masses leading to cord compression in a patient with hereditary persistence of fetal hemoglobin

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