Intracranial extramedullary hematopoiesis: MR findings with pathologic correlation.

Koch, Bernadette L.; Bisset, George S.; Bisset, R.; Zimmer, Mike

doi:10.2214/ajr.162.6.8192010

Cited by 19 publications

(19 citation statements)

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“…Intracranial extramedullary hematopoiesis is extremely rare [5,6]. The pathway of EMH involvement of the dura is unknown, but it is postulated that the dura has hematopoietic capacity in the fetus and EMH may originate from primitive rests.…”

Section: Intracranial Involvementmentioning

confidence: 99%

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Intracranial extramedullary hematopoiesis: Brief review of response to radiation therapy

et al. 2005

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Intracranial deposits of extramedullary hematopoiesis are extremely rare, and limited experience with the treatment of these lesions has been reported. Our review of the literature provides further insights regarding the clinical, radiological, and pathological behavior of these lesions and examines the available treatment strategies. Radiation therapy has proven to be an effective modality for treatment of this rare radiosensitive disease. Am. J. Hematol. 78:151-152, 2005. ª 2005 Wiley-Liss, Inc.

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Section: Intracranial Involvementmentioning

confidence: 99%

“…Others believe that dural involvement results from embolization of hematopoietic stem cells. It is unlikely to result from extrusion of bone marrow from nearby bone in the absence of bony erosions or fractures [6][7][8]. Symptoms of EMH are secondary to direct mass effect upon adjacent structures.…”

Section: Intracranial Involvementmentioning

confidence: 99%

Intracranial extramedullary hematopoiesis: Brief review of response to radiation therapy

et al. 2005

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“…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].…”

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confidence: 99%

“…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 meningioma, chloroma, granuloma, and metastasis [3][4][5].…”

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confidence: 99%

“…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]. The mechanism of EMH remains unclear, but may result from the activation of quiescent spleen and liver stem cells [7] or hematogenous spread of marrow stem cells [1,7].…”

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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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A common complication of myelofibrosis presenting as a rare finding in cerebrospinal fluid cytology

Ruíz-Cordero

Jorgensen

Krishnamurthy

et al. 2017

Diagnostic Cytopathology

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Herein, we present a rare case of intracranial extramedullary hematopoiesis (EMH) diagnosed by cerebrospinal fluid (CSF) cytology and describe the clinical presentation, radiologic, and pathologic findings. A 65 year-old man with a history of progressing primary myelofibrosis was admitted for headaches and right facial numbness. A brain MRI revealed focal abnormalities that were suspicious for leptomeningeal involvement of acute leukemia. Cytologic examination of CSF demonstrated a hypercellular specimen composed of hematopoietic cells including few blasts, as well as maturing red blood cells and granulocytic cells. The integration of morphologic findings, peripheral blood and bone marrow counts, as well as flow cytometric analysis of CSF and bone marrow, excluded leptomeningeal involvement by leukemic blasts and helped establish the diagnosis of intracranial EMH. Inclusion of EMH in the differential diagnosis of intracranial pathology in patients with known conditions predisposing them to EMH is important because recognizing this rare event has implications for treatment and prognosis.

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Intracranial extramedullary hematopoiesis: MR findings with pathologic correlation.

Cited by 19 publications

References 0 publications

Intracranial extramedullary hematopoiesis: Brief review of response to radiation therapy

Intracranial extramedullary hematopoiesis: Brief review of response to radiation therapy

Dural localization of extramedullary hematopoiesis. Report of a case

A common complication of myelofibrosis presenting as a rare finding in cerebrospinal fluid cytology

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