High-Dose Intravenous Corticosteroid Treatment in Childhood Idiopathic Myelofibrosis

Özsoylu, Şînasi; Ruacan, Şevket

doi:10.1159/000206081

Cited by 37 publications

(16 citation statements)

References 17 publications

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“…Response to steroids is seen in 5 (of 6) children with idiopathic MF. 20,21 In secondary myelofibrosis also there was improvement in 4 cases (including our case) with steroids where they were tried. 9,11,12 Treatment of underlying condition is the most important step in the management of secondary MF.…”

Section: Discussionmentioning

confidence: 54%

Secondary Myelofibrosis in Children

Naithani

Tyagi

Choudhry

2008

Journal of Pediatric Hematology/Oncology

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Myelofibrosis is a rare childhood myeloproliferative disorder. It has been reported as an associated complication of certain hematologic malignancies or as an isolated idiopathic process. We describe clinical course of 6 children diagnosed over 6 years. One child each responded well to steroid and treatment of the underlying condition. Three children died because of underlying conditions and 1 child was lost to follow up. A thorough search should be made for underlying disease when myelofibrosis is first diagnosed. Trephine biopsy though giving useful information, does not have a prognostic significance.

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Section: Discussionmentioning

confidence: 54%

Secondary Myelofibrosis in Children

Naithani

Tyagi

Choudhry

2008

Journal of Pediatric Hematology/Oncology

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“…60,61 More recently, we reported a prospective randomized study (n = 162), using the St Jude Total XI therapy protocol in which MDMP was compared versus usual corticosteroid. The event-free survival (EFS) for 3 years was found significantly higher in ALL patients treated with MDMP (n = 79) compared with those treated with conventional dose prednisolone (n = 87) during the induction-remission period (MDMP 30 to 20 mg/kg daily, orally before 9 AM at 1 to 7 and 8 to 15,17,19,21,23,25,27, and 29 d, respectively; prednisolone 2 mg/kg daily, orally for 1 to 29 d). 62 A significantly higher EFS rate was also found at 8 years in the 97 MDMP-treated ALL patients versus the 108 given conventional-dose prednisolone.…”

Section: Mdmp In Childhood Leukemia Allmentioning

confidence: 99%

“…Mega-dose methylprednisolone (MDMP) (daily 30 to 20 mg/kg in the AM, and in some circumstances up to 100 mg/kg) was used successfully for the first time in our clinic by Ö zsoylu in different hematologic diseases. [21][22][23][24][25][26][27][28][29][30][31][32][33][34] It must beemphasized that the levels of high-dose MP (including pulse MP) do not qualify as mega-doses. MDMP is administered over 10 minutes at around 6 AM orally or by the intravenous (IV) route when the steroid level is normally high and adrenocorticotropic hormone (ACTH) secretion is, therefore, minimally suppressed.…”

mentioning

confidence: 99%

Comparison of Megadose Methylprednisolone Versus Conventional Dose Prednisolone in Hematologic Disorders

Yetgin

Özsoylu²

2007

Journal of Pediatric Hematology/Oncology

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Glucocorticoids (GCs) are known for their clinically useful effects in immunologic and inflammatory disorders. Although there is a huge volume of knowledge concerning the cellular and molecular effects of GCs, statements regarding their effects in multiple diseases at variable doses are not clear-cut owing to pharmacogenetic differences. The main actions of GCs in hematologic disorders have been related to their differentiation-inducing and apoptosis-inducing effects, but modification of several steps of the hematopoietic and/or immune pathway has also been reported. In our clinic, mega-dose methylprednisolone (MDMP) has been successfully used for treatment of different hematologic diseases, such as leukemias, bone marrow failure in aplastic anemia, hypoplastic anemia, myelodysplastic syndrome, neutropenia, autoimmune diseases, and in some congenital hereditary diseases. Both clinical and experimental studies in our department revealed that MDMP was more effective than conventional dose steroids. It is interesting that MDMP can be curative in some congenital hereditary diseases such as Diamond-Blackfan syndrome. However, more research is required to clarify their roles in biology, physiology, and molecular genetics.

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“…The authors state that myelofibrosis 'fol lows an acute course with rapidly progres sive anemia and thrombocytopenia', with out taking our megadose méthylpredniso lone (MDMP) treatment into account which has been effective in idiopathic childhood cases [2] as well as adult patients (3,4]. This was brought to attention in the journal re cently [5].…”

Section: Myelofibrosismentioning

confidence: 99%