- Romiplostim-related myelofibrosis in refractory primary immune thrombocytopenia: A Case report. [Journal Article]
- MMedicine (Baltimore) 2019; 98(25):e15882
- CONCLUSIONS: This report represents the first evidence of romiplostim-induced myelofibrosis, which was associated with increased levels of bone marrow reticulin and Masson trichrome staining.
- Acquired amegakaryocytic thrombocytopenia as a rare cause of thrombocytopenia during pregnancy. [Journal Article]
- BCBMJ Case Rep 2019 Jun 21; 12(6)
- A rare case of acquired amegakaryocytic thrombocytopenia (AATP) in a 35-year-old woman who presented with anaemia and thrombocytopenia at 22 weeks gestation. The first diagnostic impression was of an…
A rare case of acquired amegakaryocytic thrombocytopenia (AATP) in a 35-year-old woman who presented with anaemia and thrombocytopenia at 22 weeks gestation. The first diagnostic impression was of an evolving aplastic anaemia; however, the patient was simultaneously diagnosed with severe vitamin B12 deficiency in the setting of vegetarianism. Once the cyanocobalamin deficiency was corrected, a repeat bone marrow biopsy revealed an isolated depletion of megakaryocytes, which suggested the diagnosis of AATP. Supportive care was provided for her anaemia and thrombocytopenia and she delivered a healthy baby girl with a normal platelet count. The patient was subsequently started on romiplostim with steady improvement in her platelet counts. This rare AATP case presentation highlights the importance of a well-structured diagnostic approach to thrombocytopenia during pregnancy and supports the successful use of thrombopoietin agonists for the management of AATP.
- Pharmacodynamics of romiplostim alone and in combination with pegfilgrastim on acute radiation-induced thrombocytopenia and neutropenia in non-human primates. [Journal Article]
- IJInt J Radiat Biol 2019 Jun 21; :1-12
- CONCLUSIONS: These results indicate that romiplostim alone or in combination with pegfilgrastim is effective at improving hematological parameters in an NHP model of ARS. This study supports further study of romiplostim as a medical countermeasure to improve primary hemostasis and survival in ARS.
- Immunomodulation in Primary Immune Thrombocytopenia: A Possible Role of the Fc Fragment of Romiplostim? [Review]
- FIFront Immunol 2019; 10:1196
- Fc fusion proteins and Fc fusion peptides or peptibodies are chimeric molecules composed of an active pharmacological protein or peptide and the Fc fragment of an immunoglobulin. The primary aim of t…
Fc fusion proteins and Fc fusion peptides or peptibodies are chimeric molecules composed of an active pharmacological protein or peptide and the Fc fragment of an immunoglobulin. The primary aim of this drug construct is to prolong the half-life of the active component. This molecular architecture is seen in drugs, such as etanercept, romiplostim, and the recombinant factor VIII (efmoroctocog alfa). A considerable number of Fc fusion proteins and peptibodies are currently in pre-clinical and clinical development. The isolated effect of the Fc fragment has been studied intensively during last years, but is still poorly understood in the clinical setting and in relation with the active drug and underlying disease. In this short review, we will propose new hypotheses of possible immunomodulatory functions of the Fc fragment of romiplostim in patients with primary immune thrombocytopenia.
- Thrombopoietin Receptor Agonists: Can These Be the Future Answer to the Deadly Thrombocytopenia in Dengue Fever? [Review]
- CCureus 2019 Apr 01; 11(4):e4361
- Dengue is considered the most prevalent mosquito-borne viral disease worldwide and sometimes turns out to be life-threatening. Thrombocytopenia is frequently observed in mild and severe cases of deng…
Dengue is considered the most prevalent mosquito-borne viral disease worldwide and sometimes turns out to be life-threatening. Thrombocytopenia is frequently observed in mild and severe cases of dengue. Severe thrombocytopenia, with a platelet count much below the normal range and hemorrhagic manifestation, is considered a fatal consequence of dengue that needs proper and timely management. The development of the dengue vaccine is quite challenging due to the existence of four different serotypes of the virus. Currently, neither a specific antiviral therapy nor a vaccine is available, and the common treatment modalities include fluid replacement therapy and platelet transfusions. Besides dengue, thrombocytopenia is correlated with many other diseases, particularly immune thrombocytopenic purpura (ITP). Thrombopoietin receptor (TPO-R) agonist, which is responsible for increasing platelet count, is a novel treatment option for chronic ITP patients. At present, two TPO-R agonists - eltrombopag and romiplostim - approved by the US Food and Drug Administration (USFDA) have been successfully used for the treatment of chronic ITP and other thrombocytopenic conditions. However, to date, only a single case study reported the use of romiplostim to enhance the platelet count in a myeloma patient suffering from dengue-associated thrombocytopenia. The objective of this review is to propose to the medical fraternity to consider using these TPO-R agonists to treat dengue hemorrhagic patients with thrombocytopenia and to conduct relevant researches to find out the usefulness of these molecules. This review is completely based on hypotheses and articles showing the positive response with romiplostim in dengue after going through a web-based search on various search engines. Furthermore, this review highlights the need for good-quality, randomized controlled trials and meta-analyses to detect the safety and efficacy of romiplostim and eltrombopag therapy for patients suffering from dengue-related thrombocytopenia.
- Switching thrombopoietin receptor agonist treatments in patients with primary immune thrombocytopenia. [Review]
- TATher Adv Hematol 2019; 10:2040620719837906
- Primary immune thrombocytopenia (ITP) is a bleeding disorder that conventionally has been treated with steroids or other immunosuppressive treatments. The introduction of thrombopoietin receptor agon…
Primary immune thrombocytopenia (ITP) is a bleeding disorder that conventionally has been treated with steroids or other immunosuppressive treatments. The introduction of thrombopoietin receptor agonists (TPO-RAs), which increase platelet production, dramatically changed the treatment landscape for ITP by providing patients with well-tolerated, long-term treatment options. Two TPO-RAs, eltrombopag and romiplostim, have been approved in the United States and European Union for the treatment of ITP. Some patients do not benefit from the first TPO-RA they receive, so it is assumed that the alternate TPO-RA would have the same outcome. However, eltrombopag and romiplostim have distinct pharmacodynamic and pharmacokinetic properties and may have different tolerability and efficacy in individual patients with ITP. Published retrospective studies showed that >75% of patients who switched to the alternate TPO-RA maintained or achieved a response with the new treatment. Notably, most patients who switched due to lack of efficacy with the first TPO-RA responded to the alternate TPO-RA, which demonstrates an absence of cross-resistance between the two drugs. Therefore, switching to the alternate TPO-RA if the first TPO-RA fails to demonstrate a response should be considered before the use of a less-preferable option.
- Thrombopoietin Receptor Agonists for Severe Thrombocytopenia after Allogeneic Stem Cell Transplantation: Experience of the Spanish Group of Hematopoietic Stem Cell Transplant. [Journal Article]
- BBBiol Blood Marrow Transplant 2019 May 29
- Persistent thrombocytopenia is a common complication after allogeneic hematopoietic stem cell transplantation (allo-SCT). Romiplostim and eltrombopag are the currently available thrombopoietin recept…
Persistent thrombocytopenia is a common complication after allogeneic hematopoietic stem cell transplantation (allo-SCT). Romiplostim and eltrombopag are the currently available thrombopoietin receptor agonists (TPO-RAs), and some studies with very small numbers of cases have reported their potential efficacy in the allo-SCT setting. The present retrospective study evaluated the safety and efficacy of TPO-RAs in 86 patients with persistent thrombocytopenia after allo-HSCT. Sixteen patients (19%) had isolated thrombocytopenia (PT), and 71 (82%) had secondary failure of platelet recovery (SFPR). TPO-RA therapy was started at a median of 127 days (range, 27 to 1177 days) after allo-SCT. The median initial and maximum administered doses were 50 mg/day (range, 25 to 150 mg/day) and 75 mg/day (range, 25 to 150 mg/day), respectively, for eltrombopag and 1 µg/kg (range, 1 to 7 µg/kg) and 5 µg/kg (range, 1 to 10 µg/kg), respectively, for romiplostin. The median platelet count before initiation of TPO-RA therapy was 14,000/µL (range, 1000 to 57,000/µL). Platelet recovery to ≥50,000/µL without transfusion support was achieved in 72% of patients at a median time of 66 days (range, 2 to 247 days). Eighty-one percent of the patients had a decreased number of megakaryocytes before treatment, showing a slower response to therapy (P = .011). The median duration of treatment was 62 days (range, 7 to 700 days). Grade 3-4 adverse events (hepatic and asthenia) were observed in only 2% of the patients. At last follow-up, 81% of patients had discontinued TPO-RAs and maintained response, and 71% were alive. To our knowledge, this is the largest series analyzing the use of TPO-RAs after allo-SCT reported to date. Our results support the efficacy and safety in this new setting. Further prospective trials are needed to increase the level of evidence and to identify predictors of response.
- Thrombopoietin receptor agonist (TPO-RA) treatment raises platelet counts and reduces anti-platelet antibody levels in mice with immune thrombocytopenia (ITP). [Journal Article]
- PPlatelets 2019 May 30; :1-4
- Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder in which autoantibodies and/or autoreactive T cells destroy platelets and megakaryocytes in the spleen and bone marrow, respectively. …
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder in which autoantibodies and/or autoreactive T cells destroy platelets and megakaryocytes in the spleen and bone marrow, respectively. Thrombopoietin receptor agonists (TPO-RA e.g. Romiplostim and Eltrombopag) have made a substantial contribution to the treatment of patients with ITP, which are refractory to first-line treatments and approximately 30% demonstrate sustained elevated platelet counts after drug tapering. How TPO-RA induce these sustained responses is not known. We analyzed the efficacy of a murine TPO-RA in a well-established murine model of active ITP. Treatment with TPO-RA (10 ug/kg, based on pilot dose escalation experiments) significantly raised the platelet counts in ITP-mice. Immunomodulation was assessed by measuring serum IgG anti-platelet antibody levels; TPO-RA-treated mice had significantly reduced IgG anti-platelet antibodies despite the increasing platelet counts. These results suggest that TPO-RA is not only an efficacious therapy but also reduces anti-platelet humoral immunity in ITP.
- Use of Romiplostim during pregnancy as a rescue therapy in primary immune thrombocytopenia: Literature review and case description. [Journal Article]
- PPlatelets 2019 May 22; :1-4
- Thrombocytopenia could appear during pregnancy, in up to 8-10% of the cases, where 3-5% is related to an autoimmune process so-called immune thrombocytopenia (ITP). We present a 34-year-old woman deb…
Thrombocytopenia could appear during pregnancy, in up to 8-10% of the cases, where 3-5% is related to an autoimmune process so-called immune thrombocytopenia (ITP). We present a 34-year-old woman debuted at 13 weeks gestation, with a platelet count of 19 × 109/L and petechiae. She did not respond to initial treatment with corticosteroids and intravenous immunoglobulin. At this point, considering the limited treatment options due to toxicity and/or teratogenesis of other drugs proven to be effective against ITP like azathioprine, rituximab, cyclophosphamide, etc. and the risk of bleeding symptoms, either from mother or fetus, we decided to begin treatment with Romiplostim (thrombopoietin receptor agonist). On reviewing the literature at this matter, only eight cases with ITP were treated during pregnancy with Romiplostim and only one of those, the ITP, was refractory to Romiplostim. In a retrospective study, Romiplostim used as a bridge to surgery in 47 patients stated a platelet count increment higher than 100 × 109/L in 79% cases after two doses of Romiplostim. According to bibliography, we decided to start Romiplostim to our patient at 35 weeks of gestation with a spectacular platelet count recovery of 158 × 109/L within 1 week of treatment, at 36th week, and after induced labor, she had on the very next day an eutocic vaginal childbearing without major bleeding complications.
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- Proposal of treatment algorithm for immune thromocytopenia in adult patients of a hematology service at a referral center in Northeastern Brazil. [Review]
- HTHematol Transfus Cell Ther 2019 Feb 16
- The management of adult (≥18 years) immune thrombocytopenia patients relies on platelet count, the risk of bleeding and presence of bleeding.
The management of adult (≥18 years) immune thrombocytopenia patients relies on platelet count, the risk of bleeding and presence of bleeding.