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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 rate.According to the etiology,this disease can be divided into acute varicose veins and nonvaricose veins.Bleeding from esophageal varices is a life-threatening complication of portal hypertension.Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10 mmHg.Cirrhosis 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 world.Primary 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 myelofibrosis.CASE SUMMARY We present a rare case of primary myelofibrosis with gastrointestinal bleeding as the primary manifestation that presented as portal-superior-splenic mesenteric vein thrombosis.Peripheral blood tests revealed the presence of the JAK2 V617F mutation.Bone 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

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Efficacy and safety of JAK inhibitor INC424 in patients with primary and post-polycythemia vera or post-essential thrombocythemia myelofibrosis in the Chinese population

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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

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Bone marrow metastatic neuroendocrine carcinoma with unknown primary site:A case report and review of the literature

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World Journal of Clinical Cases 2022年第30期10卷 11074-11081页

作者： Xue-Bing Shi Wen-Xia Deng Feng-Xiang Jin Department of Medical Oncology Tongling People’s HospitalTongling 244000Anhui ProvinceChina Department of Hematology Tongling People’s HospitalTongling 244000Anhui ProvinceChina

BACKGROUND Metastatic neuroendocrine carcinoma(NEC) of bone marrow is uncommon.Here,we report a case of bone marrow metastatic NEC with an unknown primary site.CASE SUMMARY A 73-year-old Chinese woman was admitted to our hospital because marked chest distress and asthma lasting 1 d on March 18,2018.She was initially diagnosed with pulmonary infection,cardiac insufficiency,thrombocytopenia and severe anemia.Following treatment with antibiotic therapy,diuresis and blood transfusion,the patient’s symptoms greatly improved.After bone marrow examinations,the patient was diagnosed with bone marrow metastatic NEC,bone marrow necrosis(BMN) and secondary myelofibrosis(MF).Further imaging workup did not show the primary tumor,we presumed that the primary site might regress spontaneously or merely be unexplored due to lack of positron emission tomography with gallium peptide.Everolimus(10 mg/d) was added to the treatment and the best supportive and symptomatic therapies were also administered.Unfortunately,the patient’s condition continued to deteriorate and she died on May 15,2018.CONCLUSION Bone marrow invasion of NEC is rare and our patient who suffered from bone marrow metastatic NEC as well as secondary BMN and MF had an extremely poor prognosis.Bone marrow biopsy plays an important role in the diagnosis of solid tumors invading bone marrow.

关键词： Neuroendocrine neoplasm Bone marrow metastasis Bone marrow necrosis myelofibrosis Everolimus Case report

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Risk of hepatitis B reactivation in patients with myeloproliferative neoplasms treated with ruxolitinib

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World Journal of Hepatology 2023年第11期15卷 1188-1195页

作者： Adeniyi Abraham Adesola Matei-Alexandru Cozma Yong-Feng Chen Bahadar Singh Srichawla Mihnea-Alexandru Găman Department of Medicine and Surgery Faculty of Clinical SciencesCollege of MedicineUniversity of IbadanIbadanNigeria Faculty of Medicine "Carol Davila"University of Medicine and PharmacyBucharest 050474Romania Department of Gastroenterology Colentina Clinical HospitalBucharest 020125Romania Department of Basic Medical Sciences Medical College of Taizhou UniversityTaizhou 318000Zhejiang ProvinceChina Department of Neurology University of Massachusetts Chan Medical SchoolWorcesterMA 01655United States Department of Hematology Center of Hematology and Bone Marrow TransplantationFundeni Clinical InstituteBucharest 022328Romania Cellular and Molecular Pathology Department Stefan S.Nicolau Institute of VirologyRomanian AcademyBucharest 030304Romania

Classical Philadelphia-negative myeloproliferative neoplasms(MPNs),i.e.,polycythemia vera,essential thrombocythemia,and primary/secondary myelofibrosis,are clonal disorders of the hematopoietic stem cell in which an uncontrolled proliferation of terminally differentiated myeloid cells occurs.MPNs are characterized by mutations in driver genes,the JAK2V617F point mutation being the most commonly detected genetic alteration in these hematological malignancies.Thus,JAK inhibition has emerged as a potential therapeutic strategy in MPNs,with ruxolitinib being the first JAK inhibitor developed,approved,and prescribed in the management of these blood cancers.However,the use of ruxolitinib has been associated with a potential risk of infection,including opportunistic infections and reactivation of hepatitis B.Here,we briefly describe the association between ruxolitinib treatment in MPNs and hepatitis B reactivation.

关键词： Ruxolitinib Myeloproliferative neoplasms Hepatitis B Polycythemia vera myelofibrosis JAK inhibitor

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Acute myocardial infarction in myeloproliferative neoplasms

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World Journal of Cardiology 2023年第11期15卷 571-581页

作者： Muhammad Romail Manan Vincent Kipkorir Iqra Nawaz Maryann Wanjiku Waithaka Bahadar Singh Srichawla Amelia Maria Găman Camelia Cristina Diaconu Mihnea-Alexandru Găman Services Institute of Medical Sciences Lahore 54000Pakistan Department of Human Anatomy and Physiology University of NairobiNairobi 00100Kenya Quaid-e-Azam Medical College Bahawalpur 63100Pakistan Department of Neurology University of Massachusetts Chan Medical SchoolWorcesterMA 01655United States Department of Pathophysiology University of Medicine and Pharmacy of CraiovaCraiova 200143Romania Clinic of Hematology Filantropia City HospitalCraiova 200143Romania Department of Internal Medicine Faculty of Medicine“Carol Davila”University of Medicine and PharmacyBucharest 050474Romania Internal Medicine Clinic Clinical Emergency Hospital of BucharestBucharest 105402Romania Faculty of Medicine “Carol Davila”University of Medicine and PharmacyBucharest 050474Romania Department of Hematology Center of Hematology and Bone Marrow TransplantationFundeni Clinical InstituteBucharest 022328Romania Department of Cellular and Molecular Pathology Stefan S.Nicolau Institute of VirologyRomanian AcademyBucharest 030304Romania

Myeloproliferative neoplasms(MPNs)are a heterogeneous group of hematologic malignancies characterized by an abnormal proliferation of cells of the myeloid lineage.Affected individuals are at increased risk for cardiovascular and thrombotic events.Myocardial infarction(MI)may be one of the earliest clinical manifestations of MPNs or may be a thrombotic complication that develops during the natural course of the disease.In the present review,we examine the epidemiology,pathogenesis,clinical presentation,and management of MI in MPNs based on the available literature.Moreover,we review potential biomarkers that could mediate the MI-MPNs crosstalk,from classical biochemical tests,e.g.,lactate dehydrogenase,creatine kinase and troponins,to pro-inflammatory cytokines,oxidative stress markers,and clonal hematopoiesis.

关键词： Myeloproliferative neoplasms Polycythemia vera Essential thrombocythemia myelofibrosis Myocardial infarction Acute coronary syndrome Biomarker Clonal hematopoiesis

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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

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Toxicity of targeted anticancer treatments on the liver in myeloproliferative neoplasms

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世界肝病学杂志:英文版(电子版) 2023年第9期15卷 1021-1032页

作者： Shubhrat Purwar Anam Fatima Himashree Bhattacharyya Lakshmi Venkata Simhachalam Kutikuppala Matei-Alexandru Cozma Bahadar Singh Srichawla Leah Komer Khulud Mahmood Nurani Mihnea-Alexandru Găman Department of Internal Medicine Grant Government Medical CollegeMumbai 400008MaharashtraIndia Department of Internal Medicine Pandit Jawaharlal Nehru Memorial Medical CollegeRaipur 492001ChhattisgarhIndia Department of Community&Family Medicine AIIMS GuwahatiAssam 781101India Department of General Surgery Dr NTR University of Health SciencesVijayawada 521104Andhra PradeshIndia Faculty of Medicine “Carol Davila”University of Medicine and PharmacyBucharest 050474Romania Department of Gastroenterology Colentina Clinical HospitalBucharest 020125Romania Department of Neurology University of Massachusetts Chan Medical SchoolWorcesterMA 01655United States Department of Psychiatry University of TorontoToronto M5G 1V7OntarioCanada Faculty of Health Sciences University of NairobiNairobi 30197-00100Kenya Department of Hematology Center of Hematology and Bone Marrow TransplantationFundeni Clinical InstituteBucharest 022328Romania

The liver has a central role in metabolism,therefore,it is susceptible to harmful effects of ingested medications(drugs,herbs,and nutritional supplements).Druginduced liver injury(DILI)comprises a range of unexpected reactions that occur after exposure to various classes of medication.Even though most cases consist of mild,temporary elevations in liver enzyme markers,DILI can also manifest as acute liver failure in some patients and can be associated with mortality.Herein,we briefly review available data on DILI induced by targeted anticancer agents in managing classical myeloproliferative neoplasms:Chronic myeloid leukemia,polycythemia vera,essential thrombocythemia,and myelofibrosis.

关键词： Myeloproliferative neoplasms Chronic myeloid leukemia myelofibrosis Polycythemia vera Essential thrombocythemia Hepatotoxicity Drug-induced liver injury

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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

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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

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Intestinal obstruction caused by extramedullary hematopoiesis and ascites in primary myelofibrosis

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World Journal of Gastroenterology 2014年第33期20卷 11921-11926页

作者： Xiu-Qing Wei Zong-Heng Zheng Yi Jin Jin Tao Kodjo-Kunale Abassa Zhuo-Fu Wen Chun-Kui Shao Hong-Bo Wei Bin Wu Department of Gastroenterology the Third Affiliated Hospital of Sun Yat-Sen University Department of Gastrointestinal Surgery the Third Affiliated Hospital of Sun Yat-Sen University Department of Pathology the Third Affiliated Hospital of Sun Yat-Sen University

Primary myelofibrosis(PMF) is a clonal hematopoietic stem cell disorder. It is characterized by bone marrow fibrosis, extramedullary hematopoiesis with hepatosplenomegaly and leukoerythroblastosis in the peripheral blood. The main clinical manifestations of PMF are anemia, bleeding, hepatosplenomegaly, fatigue, and fever. Here we report a rare case of PMF with anemia, small bowel obstruction and ascites due to extramedullary hematopoiesis and portal hypertension. The diagnosis was difficult to establish before surgery and the differential diagnosis is discussed.

关键词： Primary myelofibrosis Intestinal obstruc-tion Asci

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