Hydroxyurea as a first-line treatment of extramedullary hematopoiesis in patients with beta thalassemia: Four case reports

Karimi, Mehran; Cohan, Nader; Pishdad, Parisa

doi:10.1179/1607845414y.0000000168

Cited by 24 publications

(10 citation statements)

References 14 publications

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“…The successful use of hydroxyurea as monotherapy has been reported in the management of EMH masses [8–10]. These patients either had autoimmune hemolytic anemia limiting the use of transfusion [9], hemoglobin level was above 7 g/dL [11], hemoglobin level was at baseline [8], or clinical presentations involving sites other than the spinal cord lessening the urgency of mass shrinkage [12]. Hydroxyurea monotherapy was also the treatment of choice in a patient with cirrhosis when there was a concern for volume overload from transfusion [13], but the response was short lived.…”

Section: Discussionmentioning

confidence: 99%

Spinal cord compression due to extramedullary hematopoiesis in a patient with E-beta-thalassemia managed without radiation or surgery

Diatte

Gaston

et al. 2018

Journal of Community Hospital Internal Medicine Perspectives

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Extramedullary hematopoiesis (EMH) in individuals with thalassemia is often the result of undertreated severe anemia. Radiation or surgery is often the chosen approach to handle spinal cord compression due to these paraspinal EMH elements. Our patient is a 28-year-old male with E-beta-thalassemia who presented with both upper thoracic and lower extremity symptoms of spinal cord compression and was successfully managed with the combination of transfusion and hydroxyurea. Given the variation in symptoms as a result of the sporadic location as well as the extent of these EMH elements along the spinal canal, the hematological communities will continue to benefit from case reports that offer treatment therapy.

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Section: Discussionmentioning

confidence: 99%

Spinal cord compression due to extramedullary hematopoiesis in a patient with E-beta-thalassemia managed without radiation or surgery

Diatte

Gaston

et al. 2018

Journal of Community Hospital Internal Medicine Perspectives

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“…In TI patients with treatment by HU, paraspinal masses were not lower than TI patients without treatment by HU; in TI patients with treatment by HU rib widening and facial bone deformity were higher than TI patients without treatment by HU, which was not statically significant. Other studies showed that treatment with hydroxyurea could improve sense of well-being, also reduce EMH depending on genetic properties and severity of the symptoms ( 15 , 20 ).…”

Section: Discussionmentioning

confidence: 99%

“…TI patients have higher level of hemoglobin and less need for blood transfusion. Improvement of anemia by treatment with hydroxyurea is a known therapy for these patients ( 15 - 20 ). Hydroxyurea is an antimetabolite drug, which can reduce speed of skeletal changes, rib widening and erosion ( 16 , 21 ) by increasing hemoglobin F level.…”

Section: Introductionmentioning

confidence: 99%

Comparative Study of Radiographic and Laboratory Findings Between Beta Thalassemia Major and Beta Thalassemia Intermedia Patients With and Without Treatment by Hydroxyurea

Foroughi¹,

Ghaffari²,

Haghpanah³

et al. 2015

Iran Red Crescent Med J

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Background:In patients with thalassemia, chronic anemia causes bone marrow expansion and consequently skeletal manifestation in spine, skull, face and rib bones.Objectives:We aimed to compare chest radiographic findings and facial bone deformity in patients with thalassemia major (TM) and intermedia.Patients and Methods:In this cross sectional study, 86 consecutive thalassemia patients referring to the Thalassemia clinic in Shiraz, Southern Iran were evaluated during 2012. Patients were divided into three groups including TM and thalassemia intermedia (TI) with and without taking hydroxyurea (HU). Findings ofchest radiography (trabeculation, rib widening and paraspinal masses) as well as facial bone deformity were evaluated by an expert radiologist. Besides, laboratory findings were measured regarding hemoglobin, ferritin, NRBC and platelet count.Results:All radiologic findings were significantly higher in patients with TI compared to TM (P< 0.05). In patients with TI, only trabeculation was observed with a higher frequency in patients with HU compared to those without HU (68% vs. 27.3%, P= 0.008). In the regression model, from all variables evaluated, only NRBC showed a significant correlation with trabeculation (Exp B = 1.014, CI: 1.004-1.025, P = 0.008) and age showed a significant correlation with paravertebral mass (Exp B = 1.147, CI: 1.03-1.27, P = 0.013).Conclusions:In patients with TM, bone widening, trabeculation, paraspinal masses and facial bone deformity were lower than patients with TI, whichcan be related to effectiveness of therapy with blood transfusion irrespective of its adverse effects in TM patients.

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“…The blood count showed HCT 70%, Hb 22.1g/dl, and SpO2 gasometry 81%. Hydroxycarbamide was used as a first-line treatment [ 5 ], but it is not effective in controlling clinical symptoms. The patient was qualified for palliative treatment with radio-therapy as secondary treatment [ 6 ].…”

Section: Case Reportmentioning

confidence: 99%

A Case of Multifocal Extramedullary and Non-Hepatosplenic Extramedullary Hematopoiesis in a 43-Year-Old Man with a History of Congenital Eisenmenger Syndrome

Gawroński

Kruczkowska-Tarantowicz

Rzepecki

et al. 2022

Am J Case Rep

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Patient: Male, 43-year-old Final Diagnosis: Extramedullary hematopoiesis Symptoms: Pain of legs Medication: — Clinical Procedure: — Specialty: Hematology Objective: Rare disease Background: Extramedullary hematopoiesis in organs outside the bone marrow most commonly occurs in the liver and spleen. This report is of a case of multifocal extramedullary and non-hepatosplenic extramedullary hematopoiesis a 43-year-old man with a history of congenital Eisenmenger syndrome. Case Report: We present the case of a 43-year-old patient with complex heart disease and full-blown Eisenmenger syndrome associated with ventricular septal defect, bicuspid right ventricle, and pulmonary hypertension. In July 2020, the patient began to report neurological symptoms in the form of lower-limb numbness and weakness of lower-limb strength, with additional increasing lower-limb edema. A CT scan of the abdomen and pelvis revealed a 63×102×103 mm soft-tissue mass in the pelvis, located behind the urinary bladder. Due to the suspicion of proliferative disease, mainly of the lymphatic system, a diagnostic trephine biopsy was performed first, but no tumor cell infiltration was found. Then, the patient was qualified for diagnostic surgery. During the operation, tumor sections were harvested. Histopathological examination of the tissue sections showed extramedullary hematopoiesis in the tumor lining. Hydroxycarbamide was used as first-line treatment. However, it was not effective in controlling clinical symptoms. Therefore, the patient was qualified for radiotherapy as a second-line palliative treatment. Conclusions: This report presents a patient with cyanotic heart disease and extramedullary and non-hepatosplenic hematopoiesis presenting as masses that mimicked malignancy. In this case, palliative radiotherapy effectively reduced the symptoms due to the size of the mass lesion.

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Hydroxyurea as a first-line treatment of extramedullary hematopoiesis in patients with beta thalassemia: Four case reports

Abstract: HU is a good treatment option for patients with EMH and it could be a substitute for radiotherapy and invasive surgery or regular blood transfusion.

Cited by 24 publications

References 14 publications

Spinal cord compression due to extramedullary hematopoiesis in a patient with E-beta-thalassemia managed without radiation or surgery

Spinal cord compression due to extramedullary hematopoiesis in a patient with E-beta-thalassemia managed without radiation or surgery

Comparative Study of Radiographic and Laboratory Findings Between Beta Thalassemia Major and Beta Thalassemia Intermedia Patients With and Without Treatment by Hydroxyurea

A Case of Multifocal Extramedullary and Non-Hepatosplenic Extramedullary Hematopoiesis in a 43-Year-Old Man with a History of Congenital Eisenmenger Syndrome

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