Intracranial involvement in extramedullary hematopoiesis: case report and review of the literature

Haidar, Salwa; Ortiz‐Neira, Clara L.; Shroff, Manohar; Gilday, David L.; Blasér, Susan

doi:10.1007/s00247-004-1361-y

Cited by 31 publications

(26 citation statements)

References 16 publications

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“…Nearly ten cases of adult intracranial EMH have been published so far in the setting of AMM [3,10]. Headache, seizures, hemiplegia, or cranial nerve compression can reveal intracranial EMH, but it is usually asymptomatic [1,3,5]. In our patient the clinical manifestation resulted from dural compression of the neurological structures and intracranial hypertension.…”

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confidence: 70%

“…EMH appears as unique or multiple iso-or hyperintense extra-axial masses appended to the meninges with homogeneous enhancement after contrast administration [4,7]. These aspects are non-specific, and the differential diagnosis includes meningioma, chloroma, granuloma, and metastasis [3][4][5]. Beside nodular forms, EMH can also appear as a diffuse pachymeningeal thickening [4,5], and both can be combined as in our case.…”

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confidence: 78%

“…These aspects are non-specific, and the differential diagnosis includes meningioma, chloroma, granuloma, and metastasis [3][4][5]. Beside nodular forms, EMH can also appear as a diffuse pachymeningeal thickening [4,5], and both can be combined as in our case. When a specific treatment is necessary, diagnosis confirmation needs tissue biopsy and histological examination showing hematopoietic cell infiltration [3,4,7,10].…”

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confidence: 88%

“…There was no splenomegaly at clinical examination. The patient was alive and in good health 1 year later.Neurological localization of EMH is very rare and can occur in both the cranial and spinal duramater [4,5,9], while brain or spinal cord parenchyma infiltration is exceptional [3]. Nearly ten cases of adult intracranial EMH have been published so far in the setting of AMM [3,10].…”

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Dural localization of extramedullary hematopoiesis. Report of a case

et al. 2009

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Sir, Extramedullary haematopoiesis (EMH) is a compensatory mechanism of bone marrow fibrosis [1]. It is frequent with agnogenic myeloid metaplasia (AMM) and rare with other myeloproliferative disorders [1,8]. EMH is usually hepatosplenic, but nonhepatosplenic (NHS)-EMH can occur [1,8]. Among them, neurological localization is quite rare [3].A 46-year-old man was referred for horizontal diplopia, headache, and photophobia. AMM was diagnosed 2 months earlier after the fortuitous discovery of abnormal blood leukocyte count and splenomegaly, confirmed by bone marrow biopsy, and treated with 500 mg/day hydroxycarbamide. Neurological examination found a right VIth nerve palsy, left mandible hypoesthesia, and muscle weakness in the left lower limb. Tendon reflexes were normal. The patient complained of blurred vision, and fundoscopy showed papillary edema. A brain CT scan showed a 14 9 17-mm contrast-enhanced nodular lesion appended to the dura of the left temporal lobe. On MRI, this lesion enhanced after gadolinium injection on T1-weighted sequences. In addition, there was a diffuse pachymeningeal thickening, especially in the anterior regions with homogenous contrast enhancement (Fig. 1). Spinal tap revealed a high opening pressure. Cerebrospinal fluid (CSF) protein concentration was 0.86 g/l, with normal IgG. Cytological examination was normal. Chest X-ray showed bilateral pleural effusion. A surgical meningeal biopsy in the left temporal fossa showed meningeal infiltration by megakaryocytes, erythroblasts, and myeloid cells corresponding to intradural EMH. There was no blast cell excess. Cytospin examination of the pleural liquid showed a similar aspect. One month later, the patient developed acute myeloid leukemia (AML), confirmed by bone marrow aspiration. The patient was treated with intensive systemic and intrathecal chemotherapy followed by allogenic hematopoietic stem cell transplantation (HSCT). One month after treatment, there were no more neurological symptoms, and brain MRI was normal. There was no splenomegaly at clinical examination. The patient was alive and in good health 1 year later.Neurological localization of EMH is very rare and can occur in both the cranial and spinal duramater [4,5,9], while brain or spinal cord parenchyma infiltration is exceptional [3]. Nearly ten cases of adult intracranial EMH have been published so far in the setting of AMM [3,10]. Headache, seizures, hemiplegia, or cranial nerve compression can reveal intracranial EMH, but it is usually asymptomatic [1,3,5]. In our patient the clinical manifestation resulted from dural compression of the neurological structures and intracranial hypertension. The normal CSF cytology can be explained by the absence of EMH extension to the arachnoid. MRI is the diagnostic investigation of choice [4,7]. EMH appears as unique or multiple iso-or hyperintense extra-axial masses appended to the meninges with homogeneous enhancement after contrast administration [4,7]. These aspects are non-specific, and the differential diagnosis includes meningio...

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confidence: 78%

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Dural localization of extramedullary hematopoiesis. Report of a case

et al. 2009

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“…Paraspinal EMH appears as unique, multiple iso- or hyperintense masses, with homogeneous enhancement following contrast administration (23). These masses are usually lobular, well-circumscribed masses of intermediate signal intensity on T1-weighted images and low signal intensity on T2-weighted images (23,24). The CT appearance is characterized by the heterogeneous soft tissue density mass.…”

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confidence: 99%

Extramedullary hematopoiesis: A report of two cases

Zhang

Liu

et al. 2016

Experimental and Therapeutic Medicine

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Extramedullary hematopoiesis (EMH) is defined as hematopoiesis occurring in organs outside of the bone marrow. The present report describes two cases of thalassemic patients with paraspinal medullary hematopoiesis and analyzes the clinical manifestations, imaging, pathology, diagnosis and treatment of EMH. In addition, a supplementary review of previously published cases is provided along with a review of the related literature. Computed tomography (CT) of the first case revealed multiple paraspinal masses, and the largest was 6.2×8.0 cm in diameter. Likewise, CT of the second patient revealed multiple paraspinal masses in the bottom of the left thoracic cavity, and the largest was measured 10.1×10.5 cm. The two cases underwent surgical biopsy and the findings were compatible with a diagnosis of EMH. In conclusion, EMH is a compatible and rare disease, and should be distinguished from other neoplasms. EMH must considered when masses with characteristic radiologic appearance are detected in patients with thalassemia intermedia.

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Cost‐effectiveness of hydroxyurea in reducing the frequency of pain episodes and hospitalization in pediatric sickle cell disease

2010

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In a cohort of children with sickle cell disease (SCD) and vaso-occlusive pain visits served through South Carolina's Medicaid system over a 6-year period (N 5 523), we compared the number of vaso-occlusive pain or acute chest syndrome (ACS)/pneumonia episodes, and outpatient or acute service costs in those treated or not treated with hydroxyurea (HU). HU may be an underused intervention for SCD in this practice setting, for a variety of reasons. Treatment with HU varied greatly, appears to have been administered to more severely ill children, but was associated with a reduction in vaso-occlusive pain episodes, hospitalizations, and total costs of care within the HU cohort during a 2-3 year period of active HU treatment. Those receiving care through specialized SCD clinics were less likely to have pain or acute care episodes (RR 5 0.79, P < 0.0001; RR 5 0.90, P 5 0.01). Compared with the non-HU cohort, the HU group evinced a significantly higher risk of experiencing vaso-occlusive pain episodes (RR 5 3.32, P < 0.0001) and ACS/pneumonia episodes (RR 5 2.66, P < 0.0001), and higher outpatient, inpatient/emergency, and total service costs (RR 5 1.85, 2.11, 2.10, and P < 0.0001, respectively) over time. HU is clinically effective in reducing pain episodes, hospitalizations, and total care costs, but those receiving it might be more severely ill.Erythrocytes in children with sickle cell disease (SCD) become deoxygenated, dehydrated, and crescent-shaped, and tend to aggregate or stick to blood vessel walls, blocking blood flow within limbs and organs, causing painful episodes. These patients are frequently seen in emergency departments and hospitalized for these severe pain episodes [1]. SCD poses an enormous personal burden to these young patients and is also costly to the family and third-party payer, especially for low-income children [2]. Interventions designed to control vaso-occlusive pain episodes, and avoid hospitalizations may reduce the significant personal and economic burdens of the disease [3,4].Hydroxyurea (HU), a myelosuppressive agent, which raises the levels of Hb F [5] and of hemoglobin [6][7][8], effectively decreases the rate of painful vaso-occlusive and acute chest syndrome (ACS) episodes by 50% in adults [9][10][11][12], and is generally safe and well-tolerated in children older than 5 years of age [13]. There is strong evidence that HU reduces the frequency of hospitalization in children with SCD and moderate evidence that it decreases the frequency of painful crises [14]. Given the short-term safety profile of HU in children and its established efficacy in adults, HU is commonly used, off-label, in children with multiple painful episodes (3 per year) to reduce episode frequency and acute services utilization [15][16][17], even in children as young as 9 months.Results from a multicenter study of HU in sickle-cell anemia demonstrated that adult patients treated with HU had a 44% decrease in hospitalizations compared with those taking placebo, which accounted for the majority of cost savings in ...

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Intracranial involvement in extramedullary hematopoiesis: case report and review of the literature

Cited by 31 publications

References 16 publications

Dural localization of extramedullary hematopoiesis. Report of a case

Dural localization of extramedullary hematopoiesis. Report of a case

Extramedullary hematopoiesis: A report of two cases

Cost‐effectiveness of hydroxyurea in reducing the frequency of pain episodes and hospitalization in pediatric sickle cell disease

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