• Title, Summary, Keyword: cytopenia

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Treatment and response of autoimmune cytopenia occurring after allogeneic hematopoietic cell transplantation in children

  • Hwang-Bo, Seok;Kim, Seong-koo;Lee, Jae Wook;Jang, Pil-Sang;Chung, Nack-Gyun;Jeong, Dae-Chul;Cho, Bin;Kim, Hack-Ki
    • BLOOD RESEARCH
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    • v.52 no.2
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    • pp.119-124
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    • 2017
  • Background Autoimmune cytopenia (AIC) is a rare complication of allogeneic hematopoietic cell transplantation (HCT). In this study, we reviewed the diagnosis, treatment and response to therapy for pediatric patients with post-HCT AIC at our institution. Methods Of the 292 allogeneic HCTs performed from January, 2011 to December, 2015 at the Department of Pediatrics, The Catholic University of Korea, seven were complicated by post-HCT AIC, resulting in an incidence of 2.4%. Results All seven patients with post-HCT AIC had received unrelated donor transplant. Six of seven patients had a major donor-recipient blood type mismatch. The subtypes of AIC were as follows: immune thrombocytopenia (ITP) 2, autoimmune hemolytic anemia (AIHA) 2, Evans syndrome 3. Median time from HCT to AIC diagnosis was 3.6 months. All but one patient responded to first line therapy of steroid${\pm}$intravenous immunoglobulin (IVIG), but none achieved complete response (CR) with this treatment. After a median duration of treatment of 15.3 months, two patients with ITP achieved CR and five had partial response (PR) of AIC. Five patients were treated with rituximab, resulting in the following response: 2 CR, 2 PR, 1 no response (NR). Median time to response to rituximab was 26 days from first infusion. All patients are alive without event. Conclusion Post-HCT AIC is a rare complication that may not resolve despite prolonged therapy. Rapid initiation of second line agents including but not limited to B cell depleting treatment should be considered for those that fail to achieve CR with first line therapy.

Hematologic Complication of Respiratory Virus Infection (호흡기바이러스 감염에 의한 혈액학적 합병증)

  • Park, In Ho;Lee, Su Ho;You, Sung Taek;Choi, Du Young
    • Pediatric Infection and Vaccine
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    • v.20 no.3
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    • pp.178-185
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    • 2013
  • Objective: Cytopenia is a common hematologic complication of viral infections. However, information regarding hematologic effects of common respiratory virus infections is scarce. This study aimed to evaluate hematologic complications and the clinical course of patients infected with common respiratory viruses. Methods: We retrospectively analyzed 496 patients with respiratory tract infections admitted to the Department of Pediatrics, Wonkwang University Hospital from November 2011 to March 2012 using multiplex real-time polymerase chain reaction to detect the presence of respiratory viruses and hematologic abnormalities. Results: Respiratory viruses were identified in 379 patients. Respiratory syncytial virus (RSV) was most frequently detected (55.7%), followed by influenza A (Flu-A, 23.0%). Further, cytopenia was observed in 35.5% of RSV-infected patients, 25.0% of Flu-A-infected patients, and 34% of patients infected by other viruses. Each virus caused a decrease in 3 blood cell component values, which corresponded with cytopenia frequency. Of the 379 infected patients, 83 had anemia ($9.71{\pm}1.09g/dL$); 46 had neutropenia ($803.70{\pm}263.09cells/mm^3$); and 23 had transient thrombocytopenia ($142,434.78{\pm}86,835.18cells/mm^3$). However, no patient required treatment. A comparison of clinical characteristics between RSV- and Flu-A-positive patients with anemia revealed that RSV-infected patients had significantly longer duration of hospitalization. RSV was detected more commonly in young neutropenic patients, who had a shorter duration of fever. Conclusions: Our findings suggest that infections, particularly RSV and Flu-A, result in varying degrees of cytopenia, which usually improves without treatment and does not affect the clinical course of the infection.

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Spectrum of the WHO Classification De Novo Myelodysplastic Syndrome: Experience from Southern Pakistan

  • Sultan, Sadia;Irfan, Syed Mohammed;Jawed, Syeda Narisa
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.3
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    • pp.1049-1052
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    • 2016
  • Background: Myelodysplastic syndrome (MDS) is a clonal disorder of hemopoeitic stem cells, characterized by infective hematopoiesis, peripheral cytopenias along with hypercellularity of marrow and marked dysplastic features. Our aim was to study the spectrum of the WHO classification in adult Pakistani patients with MDS at disease presentation. Materials and Methods: This retrospective descriptive study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Patient data were retrieved from the maintained archives. Results: Overall, 45 patients were diagnosed at our institution with de novo MDS during the study period. There were 28 males and 17 females. Age ranged between 18 and 95 years with a mean of $57.6{\pm}17.4years$. The male to female ratio was 1.7:1. According to the WHO classification, 53.3% had refractory cytopenia with multilineage dysplasia, 22.2% had refractory cytopenia with unilineage dysplasia, 4.4% each had refractory anemia with excess of blasts-1 and II and 15.5% had MDS unclassified. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20% of patients. Hemoglobin was <10 g/dl in 41 (91.1%). Pancytopenia and bicytopenia were noted in 18 (40%) and 14 (31.1%) respectively. Conclusions: MDS in our patients presents at a relatively young age. Refractory c ytopenia with multilineage dysplasia was the dominant disease variant in our setting.

Diagnostics and Prognostication of Myelodysplastic Syndromes

  • Zini, Gina
    • Annals of Laboratory Medicine
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    • v.37 no.6
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    • pp.465-474
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    • 2017
  • MDS are a heterogeneous and complex group of clonal hematological neoplasms arising from a hematopoietic stem cell, and characterized by ineffective hematopoiesis, resulting in increased apoptosis in the bone marrow and peripheral cytopenia, which involves one or more lineages. Epigenetic changes are reported as 'founder' mutations in the case of MDS. Its incidence in the general population has been reported as five new MDS diagnoses per 100,000 people. It affects men more frequently than it does women, and its incidence increases with age. The diagnostic classification, now in use, is the one of the World Health Organization, revised in August 2016. It recognizes six distinct entities in addition to a provisional entity of childhood. In most of the cases, diagnosis is based on the morphologic quantitative and qualitative evaluation of the peripheral blood and bone marrow using basic hematological techniques. Bone marrow biopsy and flow cytometric immunophenotyping also offer support for further diagnostic elucidation, while cytogenetics and molecular genetics are presently fully integrated into prognostication, treatment processes, and decision-making.

A Case of Complete Remission of Hairy Cell Leukemia by 2-Chlorodeoxyadenosine (2-Chlorodeoxyadenosine에 의해 완전관해가 유도된 모발상 세포백혈병 1예)

  • Kim, Yong-Gil;Lee, Sang-Jin;Kim, Min-Kyung;Lee, Kyung-Hee;Hyun, Myung-Soo;Cho, Hee-Sun
    • Yeungnam University Journal of Medicine
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    • v.21 no.2
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    • pp.237-241
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    • 2004
  • Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disorder that is characterized by cytopenia, splenomegaly, and mononuclear cells displaying cytoplasmic projections. We experienced a case of hairy cell leukemia that developed in a 38-year-old man. He showed marked splenomegaly without palpable lymphoadenopathy. A complete blood cell count revealed leukopenia ($3300/{\mu}{\ell}$ with 63% of lymphocyte) and the peripheral blood smear showed abnormal lymphoid cells with cytoplasmic projections. The bone marrow smear revealed abnormal lymphocytes and severe myelofibrosis. Tartrate-resistant acid phosphatase reactivity was strongly positive in the hairy cells. The immunophenotyping results of lymphoid cells were CD5(-), CD10(-), CD19(+), CD25(+), CD103(+), CD20(+), lambda(+). The patient was treated with 2-Chlorodeoxyadenosine at a daily dose of 0.1mg/Kg by a continuous intravenous infusion for 7 days. The patient achieved complete remission.

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Neutrophil to Lymphocyte Ratio - Not an Independent Prognostic Factor in Patients with the Myelodysplastic Syndrome

  • Akinci, Sema;Silay, Kamile;Ulas, Arife;Guney, Tekin;Hacibekiroglu, Tuba;Basturk, Abdulkadir;Akinci, Muhammed Bulent;Alkan, Afra;Dilek, Imdat
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.24
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    • pp.10883-10885
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    • 2015
  • Purpose: Neutrophil-to-lymphocyte ratio (NLR) was evaluated as a potential prognostic factor in patients with myelodysplastic syndrome (MDS). Materials and Methods: Between December 2009 and April 2014, 14 female (35%) and 26 male (65%) MDS patients who were followed up in our hematology clinic were included in the study for NLR during diagnosis. Division was into two groups according to the NLR, and the correlation with mortality was evaluated. The prognostic significance of NLR regarding treatment outcome was also evaluated with adjustment for known confounding risk factors. Results: The mortality rate of the patient group was 55%, and median survival was 18 months. There was no significant correlation between mortality and NLR at a median value of 1.8 (p=0.75). Thrombocytopenia was observed to increase mortality (p=0.027), and there was a significant correlation between mortality and pancytopenia (p=0.017). Conclusions: This first study of NLR and mortality did not show any significant correlation. In centres with limited access to genetic evaluation for the presence of pancytopenia and/or thrombocytopenia at the time of diagnosis, a platelet level less than $50{\times}10^9/l$ may be poor prognostic markers in MDS patients.

Pulmonary Leukocytoclastic Vasculitis as an Initial Presentation of Myelodysplastic Syndrome

  • Lee, Seung Hyun;Kim, Jae Hyung;Park, Sejin;Won, Chang Youn;Lee, Joo-Hyun;Yi, Seong Yoon;Park, Hye Kyeong;Chang, Sun Hee;Jung, Hoon;Lee, Sung-Soon;Koo, Hyeon-Kyoung
    • Tuberculosis and Respiratory Diseases
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    • v.79 no.4
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    • pp.302-306
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    • 2016
  • Systemic vasculitis involving the lung is a rare manifestation of myelodysplastic syndrome (MDS), and secondary vasculitis is considered to have poor prognosis. A 44-year-old man presented with fever and dyspnea of 1 month duration. A chest radiograph revealed bilateral multiple wedge shaped consolidations. In addition, the results of a percutaneous needle biopsy for non-resolving pneumonia were compatible with pulmonary vasculitis. Bone marrow biopsy was performed due to the persistence of unexplained anemia and the patient was diagnosed with MDS. We reported a case of secondary vasculitis presenting as non-resolving pneumonia, later diagnosed as paraneoplastic syndrome of undiagnosed MDS. The cytopenia and vasculitis improved after a short course of glucocorticoid treatment, and there was no recurrence despite the progression of underlying MDS.

A clinical study of systemic lupus erythematosus in children (소아에서 전신성 낭창의 임상적 고찰)

  • Kim, Ji Tae;Nam, Young Mee;Lee, Jae Seung;Kim, Dong Soo
    • Korean Journal of Pediatrics
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    • v.50 no.1
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    • pp.74-78
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    • 2007
  • Purpose : Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. Methods : The data of 45 patients who were diagnosed as pediatric SLE in Severance Children's Hospital from Jan. 1996 to Dec. 2005 were analysed retrospectively. Results : The mean age at diagnosis was 10.8 (0-15) years old. And the ratio of male to female patients was 1:4. The initial manifestations were facial edema (51.1 percent), malar rash (44.4 percent), and fever (28.9 percent). The ANA (97.8 percent), anti-ds DNA antibody (82.2 percent), lupus nephritis (71.1 percent), malar rash (71.1 percent), and cytopenia (66.7 percent) were the most common findings among the classification criteria by ACR (American College of Rhematology, 1997). Conclusion : Clinical manifestations and prognosis are various in pediatric SLE. Intensive studies of SLE in children should be continued for more effective treatment.

Neoadjuvant Chemotherapy and Radiation Therapy in Advanced Stage Nasopharyngeal Carcinoma (진행된 병기의 비인강암에서의 선행보조 항암화학요법과 방사선치료)

  • Hong Semie;Wu Hong-Gyun;Park Charn II
    • Radiation Oncology Journal
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    • v.17 no.4
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    • pp.275-280
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    • 1999
  • Purpose : To assess the feasibility and the toxicity of the neoadjuvant chemotherapy on the treatment of patients with locoregionally advanced nasopharyngeal carcinoma. Methods and Materials : We analyzed 77 previously untreated and histologically confirmed advanced stage nasopharyngeal carcinoma patients treated with neoadjuvant chemotherapy followed by radiation therapy at the Seoul National University Hospital between 1984 and 1996. The stage distribution was as follows : AJCC stage III-2, stage IV-75. Sixty-six patients received infusion of 5-FU (1000 mg/m$^2$, on Day 1$\~$5) and cisplatin (100 mg/m$^2$, on Day 1), eleven patients received infusion of 5-FU (1000 mg/m$^2$, on Day 1 $\~$5) and carboplatin (300 mg/m$^2$, on Day 1) as neoadjuvant chemotherapy Prior to radiation therapy. The median follow-up for surviving patients was 44 months. Results : The overall chemotherapy response rates were 87$\%$. The toxicities of chemotherapy were mild. Only 3 patients experienced Grade 3 toxicities (1 for cytopenia, 2 for nause/vomiting). The degree of radiation induced mucositis was not severe, and ten patients developed Grade 2 mucositis. The 5-year overall survival rates were 68$\%$ and the 5-year disease free survival rates were 65$\%$. The 5-year freedom from distant metastasis rates were 82$\%$ and 5-year locoregional control rates were 75$\%$. Conclusion : This single institution experience suggests that neoadjuvant chemotherapy improves overall survival and disease free survival for patients with advanced stage nasopharyngeal carcinoma without increase of toxicity.

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Efficacy and Tolerability of Linezolid for Treatment of Infectious Spondylitis (그람양성균으로 인한 감염 척추염에서 Linezolid의 효과와 안전성)

  • Jung, Jongtak;Lee, Eunyoung;Song, Kyoung-Ho;Choe, Pyoeng Gyun;Park, Wan Beom;Bang, Ji Hwan;Kim, Eu Suk;Kim, Hong Bin;Park, Sang Won;Kim, Nam Joong;Oh, Myoung-don
    • The Korean Journal of Medicine
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    • v.93 no.5
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    • pp.464-472
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    • 2018
  • Background/Aims: Infectious spondylitis requires long-term antibiotic treatment; however, the use of intravenous antibiotics during this period has high social and monetary costs due to hospitalization. Linezolid has high oral bioavailability and is not affected by changes in renal or hepatic function. We investigated the clinical and microbiological effects of linezolid in infectious spondylitis caused by beta-lactam resistant gram-positive bacteria. Methods: Clinical data from patients who were treated with linezolid for at least four weeks were collected retrospectively from electronic medical records at the Seoul National University Hospital, Seoul National University Bundang Hospital, and Boramae Medical Center from 2006 to 2016. Results: Twenty Korean patients were treated with linezolid for at least four weeks during the study period. Of these, 14 patients were cured, four failed, and two cases of mortality occurred due to other causes than infectious spondylitis. Ten of 13 patients who had previously been assessed as vancomycin treatment failure were cured by linezolid. Bacteremia occurred in 14 patients, and 10 of these showed persistent bacteremia at the time of linezolid administration. Eight of these cases of persistent bacteremia were cured by linezolid. Median duration of linezolid treatment was 40.5 days (28-90 days). Severe cytopenia (grade II or more of National Cancer Institute criteria) was the most common adverse event, with incidences of 11.11% for neutropenia, 12.96% for anemia, and 20.37% for thrombocytopenia. Conclusions: Linezolid can be used as an effective antibiotic agent in patients with infectious spondylitis, especially when treatment failure of the first-line treatment is expected.