Cutaneous extramedullary hematopoiesis in a patient with idiopathic myelofibrosis

Miyata, Takeo; Masuzawa, Mikio; Katsuoka, Kensei; Higashihara, Masaaki

doi:10.1111/j.1346-8138.2008.00502.x

Cited by 26 publications

(43 citation statements)

References 26 publications

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“…12 Although PMF is now classified as a malignant neoplasm, involvement outside the marrow is still usually reported as EMH. 3,4 Hickling 13 described the first case of cutaneous involvement by myelofibrosis in 1937. He reported a series of patients with myelofibrosis, one of whom developed ''raised pink patches,'' especially over the abdomen.…”

Section: Discussionmentioning

confidence: 99%

“…Such involvement is usually described in the literature as ''extramedullary hematopoiesis.'' 3,4 During embryogenesis, hematopoiesis occurs mainly in the liver, with contributions from the bone marrow, thymus, spleen, and lymph nodes. EMH describes production of blood elements outside the marrow as seen in the liver during embryogenesis.…”

Section: Discussionmentioning

confidence: 99%

“…Thirty-one cases of CMF have been described in the literature, usually as EMH. 3,4 The dermal erythropoiesis seen in the ''blueberry muffin'' baby syndrome caused by intrauterine infections and hemoglobinopathies is also described in the literature as EMH but has an entirely different pathogenesis, clinical presentation, and histopathology. 5,6 EMH has also been described in adults without an underlying hematologic disorder.…”

Section: Introductionmentioning

confidence: 94%

“…However, cases of CMF continue to be reported as EMH. 3,4 CASE REPORT An 82-year-old white man presented with a 3-day history of dermal and subcutaneous nodules across his chest. His medical history was significant for a 5-year history of PMF, being treated with hydroxyurea (500 mg/day).…”

Section: Introductionmentioning

confidence: 99%

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Cutaneous Myelofibrosis With JAK2 V617F Mutation: Metastasis, Not Merely Extramedullary Hematopoiesis!

Fraga¹,

Caughron²

2010

The American Journal of Dermatopathology

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Primary myelofibrosis (PMF) is a myeloproliferative neoplasm. There are 31 reported cases of cutaneous involvement by PMF, most of which have been described as "extramedullary hematopoiesis." We report a new case of cutaneous involvement by PMF and demonstrate mutation of the Janus kinase 2 gene in the cutaneous lesions. Extramedullary myelofibrosis is best considered a metastatic phenomenon. Reporting such cases as extramedullary hematopoiesis may cause confusion with reactive forms of extramedullary hematopoiesis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 94%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Cutaneous Myelofibrosis With JAK2 V617F Mutation: Metastasis, Not Merely Extramedullary Hematopoiesis!

Fraga¹,

Caughron²

2010

The American Journal of Dermatopathology

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“…The rationale of our approach was inspired by extramedullary hematopoiesis in which the body develops hematopoietic-inductive spaces for the ectopic growth of HSPCs during states of bone marrow failure. This compensatory process is a well-recognized clinical observation that occurs in a variety of nonosseous tissues including the spleen, liver, and skin (37)(38)(39). Although extramedullary sites do not have the same microenvironment as the endogenous bone marrow, they are assumed to retain the essential elements involved in HSPC migration and engraftment.…”

Section: Discussionmentioning

confidence: 99%

Implantable microenvironments to attract hematopoietic stem/cancer cells

Lee

Milwid

et al. 2012

Proc. Natl. Acad. Sci. U.S.A.

111

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The environments that harbor hematopoietic stem and progenitor cells are critical to explore for a better understanding of hematopoiesis during health and disease. These compartments often are inaccessible for controlled and rapid experimentation, thus limiting studies to the evaluation of conventional cell culture and transgenic animal models. Here we describe the manufacture and image-guided monitoring of an engineered microenvironment with user-defined properties that recruits hematopoietic progenitors into the implant. Using intravital imaging and fluorescence molecular tomography, we show in real time that the cell homing and retention process is efficient and durable for short-and longterm engraftment studies. Our results indicate that bone marrow stromal cells, precoated on the implant, accelerate the formation of new sinusoidal blood vessels with vascular integrity at the microcapillary level that enhances the recruitment hematopoietic progenitor cells to the site. This implantable construct can serve as a tool enabling the study of hematopoiesis.

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Successful treatment of severe extremity pain in myelofibrosis with low‐dose single‐fraction radiation therapy

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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Cutaneous extramedullary hematopoiesis in a patient with idiopathic myelofibrosis

Cited by 26 publications

References 26 publications

Cutaneous Myelofibrosis With JAK2 V617F Mutation: Metastasis, Not Merely Extramedullary Hematopoiesis!

Cutaneous Myelofibrosis With JAK2 V617F Mutation: Metastasis, Not Merely Extramedullary Hematopoiesis!

Implantable microenvironments to attract hematopoietic stem/cancer cells

Successful treatment of severe extremity pain in myelofibrosis with low‐dose single‐fraction radiation therapy

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