No abstract available.
Extracorporeal Membrane Oxygenation* ; Hematologic Neoplasms* ; Humans

Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.
Acute Kidney Injury ; Anemia, Hemolytic ; Comorbidity ; Diagnosis ; Diagnosis, Differential ; Disseminated Intravascular Coagulation ; Enterohemorrhagic Escherichia coli ; Hemolytic-Uremic Syndrome ; Humans ; Hypertension, Malignant ; Mortality ; Plasma Exchange ; Plasmapheresis ; Polymerase Chain Reaction ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia ; Thrombospondins ; Thrombotic Microangiopathies

Diagnosis of thrombotic microangiopathy (TMA) is challenging due to its close association with other forms of microangiopathic hemolytic anemia, such as malignant hypertension and disseminated intravascular coagulation, and because other manifestations including cytopenia and acute kidney injury are manifestations of other medical comorbidities. Further challenges for accurate diagnosis include distinguishing between primary and secondary TMA, as well as between hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). TTP is typically differentiated from HUS by the presence of more severe thrombocytopenia, along with a higher frequency of altered mental status with relatively preserved renal function. However, the clinical course can vary among patients, requiring polymerase chain reaction testing of patient stools for enterohemorrhagic Escherichia coli and a disintegrin and metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS13) assay. To reduce the mortality rate, prompt initiation of plasmapheresis is important in cases where TPP cannot be excluded. Future advances enabling more rapid testing for ADAMTS13 levels will reduce the need for unnecessary plasmapheresis, so that treatment strategy can be more optimized.

Increasing knowledge of the molecular features of myeloproliferative neoplasms (MPNs) is being combined with existing prognostic models based on clinical, laboratory, and cytogenetic information. Mutation-enhanced international prognostic systems (MIPSS) for polycythemia vera (PV) and essential thrombocythemia (ET) have improved prognostic assessments. In the case of overt primary myelofibrosis (PMF), the MIPSS70 and its later revisions (MIPSS70+ and MIPSS70+ version 2.0) effectively predicted the overall survival (OS) of patients. Because post-PV and post-ET myelofibrosis have different biological and clinical courses compared to overt PMF, the myelofibrosis secondary to PV and ET-prognostic model was developed. Although these molecular-inspired prognostic models need to be further validated in future studies, they are expected to improve the prognostic power in patients with MPNs in the molecular era. Efforts are being made to predict survival after the use of specific drugs or allogeneic hematopoietic stem cell transplantation. These treatment outcome prediction models enable the establishment of personalized treatment strategies, thereby improving the OS of patients with MPNs.

Pericardial drainage is an important diagnostic and therapeutic option in the symptomatic patient with large amount of pericardial effusion (PE). However, when the amount of PE is relatively small, physicians are often reluctant to perform the invasive drainage of the fluid due to the increased risk of causing myocardial injury during the procedure. Even in some cases of suspected pericarditis with small amount PE, an initial empirical anti-inflammatory therapy is often recommended. A 65-year-old woman presented with mild dyspnea for two weeks. The echocardiography revealed small amount of PE. A careful fluoroscopy-guided pericardiocentesis, subsequent pericardial fluid cytology, and thorough whole body check-up demonstrated adenocarcinoma with no proven primary site. After the palliative chemotherapy, she had survived for 15 months until her death due to asphyxia. Although pericardiocentesis is considered dangerous in small amount of PE, a prompt and careful drainage may provide early detection of hidden malignancy and better survival outcome.
Adenocarcinoma* ; Aged ; Asphyxia ; Drainage ; Drug Therapy ; Dyspnea ; Echocardiography ; Female ; Humans ; Pericardial Effusion* ; Pericardial Fluid ; Pericardiocentesis* ; Pericarditis

Persistent bone marrow aplasia after intensive chemotherapy is uncommon, but is one of the fatal complications in patients with acute myeloid leukemia (AML). Although allogeneic hematopoietic stem cell transplantation (HSCT) is considered to be contraindicated for patients who have hematologic diseases with serious infections, such as bacterial septicemia or invasive fungal diseases, combined with prolonged neutropenia due to frequent morbidity and mortality, such risks can be overcome by non-myeloablative conditioning and best supportive care. Here, we report an AML patient with persistent marrow aplasia after induction therapy, treated successfully with reduced-intensity allogeneic HSCT despite severe bacterial and fungal infections.
Anemia, Aplastic ; Bone Marrow* ; Drug Therapy* ; Hematologic Diseases ; Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Myeloid, Acute* ; Mortality ; Neutropenia ; Sepsis ; Stem Cell Transplantation* ; Stem Cells*

Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome characterized by micro-angiopathic hemolytic anemia, thrombocytopenia, fever, renal disorders, and neurological manifestations. Its clinical course is rapid and the mortality rate is high if untreated or relapse occurs. Previous studies reported that rituximab, a monoclonal antibody for CD20 surface antigen on B lymphocytes, may be effective in treating idiopathic TTP that is refractory to plasma exchange or relapses after remission. A 27-year-old Vietnamese man presented with fever and fatigue starting 3 days earlier, which was diagnosed as idiopathic TTP. To overcome his poor responsiveness to combined therapy using steroids and plasma exchange, rituximab was considered. In the current case, the patient was treated with a lower dose of rituximab, instead of the conventional 375 mg/m2/week, and achieved successful remission.
Adult ; Anemia, Hemolytic ; Antigens, Surface ; Asian Continental Ancestry Group ; B-Lymphocytes ; Fatigue ; Fever ; Glucocorticoids ; Humans ; Mortality ; Neurologic Manifestations ; Plasma Exchange ; Plasma* ; Purpura, Thrombotic Thrombocytopenic* ; Recurrence ; Steroids ; Thrombocytopenia ; Thrombotic Microangiopathies ; Rituximab

Epidemiologically, multiple myeloma (MM) is a malignant disorder of plasma cells with a higher incidence among Western populations than among Asians. However, there is growing evidence of a recent increase in the age-standardized incidence rate (ASR) of MM in Asian countries, particularly Korea. Application of novel agents has resulted in significant improvement of treatment outcomes, and the advances are ongoing with the recent introduction and U.S. Food and Drug Administration’s approval of newer agents, including carfilzomib, ixazomib, elotuzumab, and daratumumab. In concert with the technical advances in the cytogenetic and molecular diagnostics of MM, modifications of its diagnosis and staging system have been attempted for better risk stratification. The modified diagnostic criteria from the International Myeloma Working Group in 2014 enabled a strategy of more active treatment for some patients with smoldering MM, with an ultra-high risk of progression, and fine-tuned the definition of end-organ damage, known as CRAB (hypercalcemia, renal insufficiency, anemia, and bone lesions). Considering Korea’s trend of aging at an unprecedented rate, we can expect that the ASR of MM will maintain a gradual increase for many years to come; therefore, MM will be a cancer of critical importance from both medical and socioeconomic perspectives in Korea.
Aging ; Anemia ; Asian Continental Ancestry Group ; Cytogenetics ; Diagnosis ; Epidemiology ; Humans ; Incidence ; Korea ; Multiple Myeloma* ; Neoplasm Staging ; Pathology, Molecular ; Plasma Cells ; Renal Insufficiency