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Frontiers of Medicine 2016年第4期10卷 437-443页

作者： Xin Du Daobin Zhou Department of Hematology Guangdong General Hospital Guangdong Academy of Medical Sciences Cruangzhou 510080 China Department of Hematology Peking Union Medical College Hospital Beijing 100730 China

A phase II study （A2202） was performed to evaluate the efficacy and safety of JAK inhibitor ruxolitinib in 63 Chinese MF patients. Ruxolitinib was given twice a day （bid） at a starting dose of 15 mg （n = 25） or 20 mg （n = 38） based on a baseline platelet count. About 94.7% of the patients achieved a reduction in spleen size, 27.0% of which exhibited significant reduction （≥35%） at week 24. Significant improvement in debilitating constitutional symptoms, as assessed by MFSAF v2.0, was observed in patients treated with ruxolitinib. Ruxolitinib treatment was generally weli tolerated by Chinese patients. Although the treatment was associated with an increase in certain adverse events （AEs） that were established as identified risks （anemia and thrombocytopenia）, these AEs were considered manageable in this clinical setting. Ruxolitinib provided substantial reductions in splenomegaly and improvements in symptoms, and was well-tolerated by Chinese patients with MF.

关键词： JAK ruxolitinib Chinese patients myelofibrosis

Acute upper gastrointestinal bleeding due to portal hypertension in a patient with primary myelofibrosis:A case report

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World Journal of Clinical Cases 2024年第15期12卷 2621-2626页

作者： Yu Chen Bing-Bing Kong He Yin Hao Liu Sheng Wu Ting Xu Department of Emergency Beijing Tsinghua Changgung HospitalSchool of Clinical MedicineTsinghua UniversityBeijing 102218China Department of Pathology Beijing Tsinghua Changgung HospitalSchool of Clinical MedicineTsinghua UniversityBeijing 102218China

BACKGROUND Acute upper gastrointestinal bleeding is a common medical emergency that has a 10%hospital mortality *** to the etiology,this disease can be divided into acute varicose veins and nonvaricose *** from esophageal varices is a life-threatening complication of portal *** hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10 *** is the most common cause of portal hypertension,and thrombosis of the portal system not associated with liver cirrhosis is the second most common cause of portal hypertension in the Western *** myeloproliferative disorders are the main cause of portal venous thrombosis,and somatic mutations in the Janus kinase 2 gene(JAK2 V617F)can be found in approximately 90% of polycythemia vera,50% of essential thrombocyrosis and 50% of primary *** SUMMARY We present a rare case of primary myelofibrosis with gastrointestinal bleeding as the primary manifestation that presented as portal-superior-splenic mesenteric vein *** blood tests revealed the presence of the JAK2 V617F *** marrow biopsy ultimately confirmed the diagnosis of myelofibrosis(MF-2 grade).CONCLUSION In patients with acute esophageal variceal bleeding due to portal hypertension and vein thrombosis without cirrhosis,the possibility of myeloproliferative neoplasms should be considered,and the JAK2 mutation test should be performed.

关键词： Acute esophageal variceal bleeding Portal hypertension myelofibrosis JAK2 V617F mutation Case report

Pseudohyperkalemia with myelofibrosis after Splenectomy

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Chinese Medical Sciences Journal 2016年第4期31卷 258-260页

作者： Sheng-li Liu Lu-wei Zhang Jun Tian Center of Kidney Transplantation and Hemodialysis Qilu Hospital of Shandong University

SEUDOHYPERKALEMIA refers the serum potassiumlevel is higher than plasma potassium due tovarious reasons in vitro monitoring, but actualpotassium level in vivo is at the normal range. Aseries of inappropriate clinical interventions due to initialfailure to recognize it may actually decrease potassiumlevel，which could cause life-threatening condition. Here wereport a patient with myelofibrosis who occurred spurioushyperkalemia after splenectomy, aiming at emphasizingthe importance of recognizing pseudodyperkalemia.

关键词： pseudohyperkalemia serum potassium plasma potassium myelofibrosis splenectomy

myelofibrosis: Prognostication and cytoreductive treatment

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World Journal of Hematology 2014年第3期3卷 85-92页

作者： Margherita Maffioli Domenica Caramazza Barbara Mora Michele Merli Francesco Passamonti Division of Hematology Department of MedicineUniversity Hospital Ospedale di Circolo e Fondazione Macchi21100 VareseItaly

Myeloproliferative neoplasms include three diseases: polycythemia vera, essential thrombocythemia and primary myelofibrosis(PMF), currently diagnosed according to the 2008 World Health Organization criteria. Patients with PMF may encounter many complications, and, among these, disease progression is the most severe. Concerning prognostication of myelofibrosis(MF), the International Prognostic scoring system(IPSS)(International Prognostic Scoring System) model at diagnosis and the Dynamic IPSS(DIPSS) anytime during the course of the disease may be useful to define survival of MF patients. The IPSS and the DIPSS are based on age greater than 65 years, presence of constitutional symptoms, hemoglobin level less than 10 g/d L, leukocyte count greater than 25 × 109/L, and circulating blast cells 1% or greater. Cytogenetic profile and mutational analysis seem to be the next step to implement MF prognostication. Concerning treatments, hydroxyurea has been considered until now the drug of choice when an anti-myeloproliferative effect is needed, but recent data on JAK inhibitors demonstrated a significant effect of these drugs on splenomegaly and symptoms.

关键词： myelofibrosis Prognosis JAK2 Ruxolitinib Fedratinib Momelotinib

Portal hypertension secondary to myelofibrosis with myeloid metaplasia:A study of 13 cases

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World Journal of Gastroenterology 2009年第25期15卷 3128-3133页

作者： Mohannad Abu-Hilal Jayant Tawaker Department of Internal Medicine Division of GastroenterologyMayo Clinic College of MedicineRochester

AIM:To describe the clinical presentation and complications of portal hypertension (PH) secondary to myelofibrosis with myeloid metaplasia (MMM).METHODS: Medical records for 123 patients with MMM were ***: Thirteen patients with PH secondary to MMM were identified. Median ages at time of MMM and PH diagnosis were 61 and 66 years, respectively. The interval from MMM diagnosis to presentation with one of the PH features ranged from 1 to 11 years. Variceal bleeding and ascites were the most common presentations. Of the eight patients who presented with variceal bleeding, six patients underwent endoscopic variceal ligation (EVL) with no variceal recurrence or hematological worsening during a 12-mo follow up ***: Patients with MMM might develop PH. Exact mechanisms leading to PH in MMM are still controversial. As in other etiologies, variceal bleeding and ascites are the most common presentations. Anemia may correlate with, and/or predict, the severity of the PH presentation in these patients. EVL can successfully control variceal bleeding in MMM. Further clinical studies are required.

关键词： Portal hypertension myelofibrosis Myeloid metaplasia Ascites Variceal bleeding

Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension

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World Journal of Gastroenterology 2012年第28期18卷 3770-3774页

作者： Koichi Tokai Hiroyuki Miyatani Yukio Yoshida Shigeki Yamada Department of Digestive Organs Jichi Medical University Saitama Medical Center Saitama 330-8503 Japan Department of Pathology Jichi Medical University Saitama Medical Center Saitama 330-8503 Japan

A 75-year old man had been diagnosed at 42 years of age as having polycythemia vera and had been monitored at another hospital. Progression of anemia had been recognized at about age 70 years, and the patient was thus referred to our center in 2008 where secondary myelofibrosis was diagnosed based on bone marrow biopsy findings. Hematemesis due to rupture of esophageal varices occurred in January and February of 2011. The bleeding was stopped by endoscopic variceal ligation. Furthermore, in March of the same year, hematemesis recurred and the patient was transported to our center. He was in irreversible hemorrhagic shock and died. The autopsy showed severe bone marrow fibrosis with mainly argyrophilic fibers, an observation consistent with myelofibrosis. The liver weighed 1856 g the spleen 1572 g, indicating marked hepatosplenomegaly. The liver and spleen both showed extramedullary hemopoiesis. myelofibrosis is often complicated by portal hypertension and is occasionally associated with gastrointestinal hemorrhage due to esophageal varices. A patient diagnosed as having myelofibrosis needs to be screened for esophageal/gastric varices. myelofibrosis has a poor prognosis. Therefore, it is necessary to carefully decide the therapeutic strategy in consideration of the patient's concomitant conditions, treatment invasiveness and quality of life.

关键词： myelofibrosis Portal hypertension Rupture of esophageal varices

A New Observation of Bone Marrow Involvement by Diffuse Large B-Cell Lymphoma Mimicking myelofibrosis

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Open Journal of Blood Diseases 2022年第2期12卷 39-44页

作者： Fall Seynabou Alioune Badara Diallo Dibor Niang El Hadji Daouda Niang Khadim Sarr Marième Lolita Camara Abibatou Sall Fatou Samba Ndiaye Department of Hematology Cheikh Anta Diop University Dakar Senegal Department of Pathology Gaston Berger University Saint-Louis Senegal

Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma, characterized by high clinical and biological heterogeneity. Patients typically present with progressive lymphadenopathy, extranodal disease and may also experience fever, night sweats and unexplained weight loss. We report here the case of a 16-year-old female with osteoarticular pain, dizziness, and dyspnea on exertion. Clinical examination showed no lymphadenopathy. Complete blood count (CBC) revealed pancytopenia and marrow smears found to be hypocellular. Initial diagnosis favored secondary myelofibrosis. Diagnosis of bone marrow involvement by DLBCL was retained on bone marrow histology and immunohistochemistry which showed infiltration of large B lymphoid cells. The patient was treated by immunochemotherapy R-CHOP regimen. This case highlights a very rare and atypical circumstance of discovery of DLBCL with myelofibrosis as an initial symptom. Prognosis value of this presentation and management difficulties are also discussed.

关键词： Diffuse Large B-Cell Lymphoma myelofibrosis Rituximab

Hyperinsulinemia and insulin resistance in a patient with type 2 diabetes complicated with myelofibrosis

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World Journal of Diabetes 2012年第8期3卷 156-157页

作者： Hidetaka Hamasaki Shuichi Mishima Hidekatsu Yanai Department of Internal Medicine Kohnodai Hospital National Center for Global Health and Medicine Chiba 272-8516 Japan

Inflammation induces insulin resistance and hyperinsulinemia due to elevation of serum cytokines such as tumor necrosis factor-α and interleukins. Chronic myeloproliferative diseases including myelofibrosis show higher serum interleukin levels than healthy subjects, which has been suggested to be the useful markers for disease activity. However, an association between myelofibrosis and insulin resistance has not ever been discussed anywhere. Here we report a case of type 2 diabetes showing remarkable hyperinsulinemia and insulin resistance possibly due to myelofibrosis.

关键词： Cytokines Hepatosplenomegaly Hyperinsulinemia Insulin resistance myelofibrosis

An exceptional case of myelodysplastic syndrome with myelofibrosis following combination chemotherapy for squamous cell lung cancer

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Cancer Biology & Medicine 2013年第2期10卷 117-120页

作者： Yi-Hao Wang Rong Fu Zong-Hong Shao Department of Hematology Tianjin Medical University General Hospital

A 60-year-old woman with squamous cell carcinoma in the right lung was successfully treated with four cycles of combination chemotherapy after surgery, and complete remission was achieved. However, the patient developed myelodysplastic syndrome (MDS) RAEB-2 with myelofibrosis after remission, possibly because of chemotherapy or DNA methylation. The patient responded well to dacitabine (Dacogen), suggesting that DNA hypomethylation agents can be a promising therapy to retard the progression of a second tumor or carcinoma.

关键词： DNA methylation myelodysplastic syndrome myelofibrosis squamous cell lung cancer

Leukocytosis at Diagnosis in Patients with Essential Thrombocythemia Is a Risk Factor for Transformation into myelofibrosis

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Open Journal of Blood Diseases 2012年第3期2卷 46-50页

作者： Irene Bertozzi Fabiana Tezza Emanuela Bonamigo Fabrizio Fabris Maria Luigia Randi Dept of Medical and Surgical Sciences University of Padua Italy

myelofibrosis (MF) represents the major long-term complication of essential thrombocythemia (ET). There is evidence that leukocytosis at diagnosis is associated with poorer survival in patients with ET. In this study, we retrospectively evaluated 143 patients with ET, diagnosed in agreement with WHO criteria, followed in a single centre over >10 years. Nine of them transformed into MF (post-essential thrombocythemia-myelofibrosis PET-MF). We compared PET-MF data at diagnosis with that of the remaining 134 patients (ET-1) and with a selected sub-group of ET-1 (ET-2, 19 pats) sex, age and follow-up duration matched to PET-MF. The PET-MF evolution rate was 4.6 per 1000 person-years;white blood cells count (WBC) count, haemoglobin levels and hematocrit were higher in PET-MF than in ET-1 (P = 0.01) while only WBC was higher than in ET-2 (P = 0.01). With multivariate analysis, only WBC count retained its signifi-cance. Our study highlights the prognostic relevance of leukocytosis on myelofibrotic transformation of ET.

关键词： Essential Thrombocythemia Leukocytosis Post-Thrombocythemia myelofibrosis Myeloproliferative Neoplasms