Advances in research related to severe pertussis in infants and young children / 中华实用儿科临床杂志

Pertussis is a highly contagious respiratory disease.Although widespread vaccination has greatly reduced the incidence of pertussis, there was a " recurrence of pertussis" in the past 30 years, and pertussis outbreaks occurred in some areas.Infants who have not been vaccinated or have not completed the full course of immunization suffer from more severe pertussis infections.Because of the atypical symptoms of young infants, missed diagnosis and misdiagnosis often occur, and pertussis cannot be diagnosed and treated in time.As a result, they can easily develop into severe pertussis or even die.In this article, recently published research on severe pertussis are summarized, so as to provide guidance for the clinical diagnosis, treatment, prevention and basic scientific research of severe pertussis.

A case of paraneoplastic hyperleukocytosis closely mimicking chronic neutrophilic leukemia

Leukemoid reaction and myeloproliferative syndrome are close mimickers and frequently pose a diagnostic dilemma, particularly when the leukocyte count is very high. Leukocyte alkaline phosphatase score frequently aids in diagnosis but may or may not be contributory, especially in differentiating chronic neutrophilic leukemia. Herein, we document a case of leukemoid reaction with extensive hyperleukocytosis in a 46-year-old female with poorly differentiated carcinoma. The tumor itself as well as the associated leukocytosis portends a poor prognosis

Advances in the diagnosis of severe pertussis and in the research of mortality risk factors from pertussis / 中华实用儿科临床杂志

Pertussis is an acute highly contagious respiratory disease,which is associated with high rate of hospitalization and death in infants and young children.The pathogenesis of severe pertussis has not been elucidated,and there has not been a consistent universal definition in severe pertussis.This article summarizes the present clinical definitions of severe pertussis related diagnosis,and the risk factors for death from pertussis.This text could benefit the clinical practice and the further researches,and to contribute to establish a regulation of the diagnosis and treatment of severe pertussis early.

Advances in the diagnosis of severe pertussis and in the research of mortality risk factors from pertussis / 中华实用儿科临床杂志

Pertussis is an acute highly contagious respiratory disease, which is associated with high rate of hospitalization and death in infants and young children.The pathogenesis of severe pertussis has not been elucidated, and there has not been a consistent universal definition in severe pertussis.This article summarizes the present clinical de-finitions of severe pertussis related diagnosis, and the risk factors for death from pertussis.This text could benefit the clinical practice and the further researches, and to contribute to establish a regulation of the diagnosis and treatment of severe pertussis early.

Transformación leucémica en paciente con linfoma folicular e hiperleucocitosis al diagnóstico / Leukemic transformation in a patient with follicular lymphoma and hyperleukocytosis at diagnosis

La fase leucémica como presentación de un linfoma folicular es rara y debe ser considerada factor de mal pronóstico. Por otra parte, la asociación entre linfoma folicular y síndrome mielodisplásico no se ha descrito. Se presenta el caso de una paciente en la que se detectó marcada leucocitosis y a la que se diagnosticó un linfoma folicular. Recibió quimioterapia con R-CHOP y FCR cuando recayó. Meses después, se realizó un aspirado medular en el cual se observaron cambios compatibles con mielodisplasia, únicamente recibió terapia de soporte y finalmente evolucionó a leucemia mieloide aguda. Aunque se conoce que la mielodisplasia puede ser secundaria al uso de quimioterapia, la paciente presentó además trisomía del cromosoma 11, descrita previamente en mielodisplasia y linfoma tipo Burkitt, la cual pudiera estar en relación con la evolución a leucemia mieloide aguda(AU)

Follicular lymphoma rarely presents with a leukemic phase and this should be considered a negative prognostic factor. Also, follicular lymphoma and myelodysplastic syndrome association has not been previously reported. Herein we present a patient who debuted with marked hyperleukocytosis and was diagnosed with follicular lymphoma, receiving CHOP-R and FCR after she relapsed. Several months later, secondary myelodysplastic changes were observed in her bone marrow. She received supportive therapy and finally progressed into acute myeloid leukemia. Although secondary myelodysplasia is known to be produced by chemotherapy, this patient additionally had trisomy 11, previously described in myelodysplasia and Burkitt's lymphoma, which could be linked to progression to acute myeloid leukemia(AU)

Características del Primer Hemograma, al Momento del Diagnóstico de Leucemia Linfoblástica Aguda, en los Niños del Instituto del Cáncer Solca-Cuenca / Characteristics of the First Hemogram, at the Time of Diagnosis of Acute Lymphoblastic Leukemia, in Children of the Solca-Cuenca Cancer Institute

ANTECEDENTES: La Leucemia Linfoblástica Aguda (LLA) es el tipo de cáncer más común en los niños, que afec-ta la sangre y la médula ósea como consecuencia de la proliferación clonal anormal de un grupo celular y que, a diferencia de lo que se cree, no siempre debuta con leucocitosis (Glóbulos Blancos> 10.000).MÉTODOS: Estudio de tipo descriptivo en el cual fueron evaluados los hemogramas de un total de 110 pacien-tes con LLA, de 1 a 18 años de edad, tratados en la So-ciedad de Lucha Contra el Cáncer (SOLCA) Cuenca, entre enero de 2009 y abril de 2014. Se analizó sus ca-racterísticas demográficas (edad, sexo, procedencia) así como su número de glóbulos blancos (GB), hemog-lobina (Hb), plaquetas, niveles de deshidrogenasa lác-tica (DHL), grupo de riesgo, presencia de cromosoma Philadelphia e inmunofenotipo (T o B).RESULTADOS: El 53% de los pacientes fueron niños. La edad promedio fue de 7.9 años. La mayor parte de ni-ños atendidos pertenecieron a la provincia del Azuay. El 50% de los pacientes presentaron leucocitosis al momento del diagnóstico, el 42% Hb menor de 8g/dL y el 80% plaquetas menores a 150 000/L. La totalidad de los niños (100%) tuvieron una DHL por encima del valor nor-mal (234 U/L). El rango de GB estuvo entre 600 y 556 000 GB/L siendo el promedio de 48 000 GB/L. El 95% presen-tó un inmunofenotipo B y el 5% de los pacientes fueron positivos para cromosoma Philadelphia. CONCLUSIONES: Tras el análisis se concluyó que la mi-tad de los pacientes no presentaron leucocitosis. La mayor parte presentó trombocitopenia y anemia. Esta información debe ser considerada al momento de re-visar un hemograma de un niño, ya que como se ha demostrado y a diferencia de lo que se pensaba, la leucocitosis no siempre está presente en la leucemia.

BACKGROUND: The acute lymphoblastic leukemia (ALL) is the most common type of childhood cancer that affects the blood and bone marrow as a result of ab-normal clonal proliferation of a cell group, and contrary to what is popularly believed, it not always debuts with leukocytosis (white blood cells> 10,000).METHODS: it is a descriptive study in which they were evaluated the blood counts from a total of 110 pa-tients with ALL, from 1 to 18 years, treated at the Society for Fight against Cancer (SOLCA) Cuenca, between January 2009 and April 2014. Their demographic cha-racteristics (age, sex, origin) and its number of white blood cells (WBCs), hemoglobin (Hb), platelet, levels of lactate dehydrogenase (LDH), risk group, presence of Philadelphia chromosome and immunophenotype (T or B) were analyzed.RESULTS: The 53% of the patients were children. The ave-rage age was 7.9 years. Most served children belonged to the Azuay province. The 50% of patients had leuko-cytosis at the moment of diagnosis, the 42% Hb less than 8g / dL and 80% lower than 150 000 platelets / L. All children (100%) had a LDH above the normal value (234 U / L). The range was between 600 and 556000 GB/ L with an average of 48 000 GB / L. The 95% presented an im-munophenotype B and 5% of patients were positive for Philadelphia chromosome.CONCLUSIONS: After the analysis it was concluded that half of the patients had leukocytosis. Most presented thrombocytopenia and anemia. This information should be considered when reviewing a child's blood count, because as has been shown and unlike what was thou-ght, leukocytosis is not always present in leukemia.

Therapeutic Leukapheresis for the Management of Hyperleukocytosis: Ten-year Experience in a Tertiary Care Hospital

BACKGROUND: Hyperleukocytosis is a medical emergency that is characterized by increased blood viscosity and predisposition to various neurological, pulmonary, and gastrointestinal complications. In addition, patients are at risk of the tumor lysis syndrome because of the increased tumor burden. Therapeutic leukapheresis is an important treatment for these emergent states. In this study, we retrospectively analyzed therapeutic leukapheresis procedures that were performed in our institution during the last 10 yr. METHODS: We retrospectively analyzed therapeutic leukapheresis procedures conducted from July 2005 to March 2015 at a tertiary care hospital. We present our observations, especially the procedural characteristics and hematological parameters before and after the aforementioned procedures. RESULTS: Seventy-two patients underwent a total of 146 therapeutic leukapheresis procedures. The average presenting white blood cell (WBC) count was 268×10(3)/µL, and ranged from 54×10(3)/µL to 673×10(3)/µL. After an average of two sessions, a statistically significant drop in the WBC counts was observed. The average WBC removal rates during the initial and entire therapeutic leukapheresis procedures of each patient were 33% and 46%, respectively. The platelet count and hemoglobin concentration were significantly reduced. CONCLUSIONS: Therapeutic leukapheresis significantly reduces peripheral WBC counts and is a safe and effective procedure for the treatment of hyperleukocytosis.

Childhood acute lymphoblastic leukemia with hyperleukocytosis at presentation

BACKGROUND: Hyperleukocytosis caused by acute lymphoblastic leukemia (ALL) is associated with early morbidity and mortality due to hyperviscosity arising from the excessive number of leukocytes.This study was designed to assess the incidence of hyperleukocytosis, survival outcomes, and adverse features among pediatric ALL patients with hyperleukocytosis. METHODS: Between January 2001 and December 2010, 104 children with previously untreated ALL were enrolled at the Pusan National University Hospital. All of them were initially stratified based on the National Cancer Institute (NCI) risk; 48 (46.2%) were diagnosed with high-risk ALL. The medical charts of these patients were retrospectively reviewed. RESULTS: Twenty (19.2%) of the 104 children with ALL had initial leukocyte counts of >100x10(9)/L, and 11 patients had a leukocyte count of >200x10(9)/L. Male gender, T-cell phenotype, and massive splenomegaly were positively associated with hyperleukocytosis. Common early complications during induction therapy included renal dysfunction, and central nervous system hemorrhage. The complete remission (CR) rate for the pediatric ALL patients with hyperleukocytosis (94.1%) was similar to the overall CR rate (95.6%). The estimated 3-year event free survival (EFS) and overall survival of ALL children with hyperleukocytosis were 75.0% and 81.2%, respectively. However, patients with initial leukocyte counts >200x10(9)/L had a lower EFS than those with initial leukocyte counts 100-200x109/L (63.6% vs. 100%; P=0.046). CONCLUSION: The outcome of pediatric ALL cases with an initial leukocyte count >200x10(9)/L was very poor, probably due to early toxicity-related death during induction therapy.

Clinical Effects and Safety of Therapeutic Leukapheresis in Hyperleukocytosis / 대한수혈학회지

BACKGROUND: Therapeutic leukapheresis is the cytoreduction procedure performed before chemotherapy in patients with hyperleukocytosis for prevention of complication. However, there have been clinical concerns about bleeding tendency due to anticoagulant used during the procedure. The aim of our study was to compare the clinical characteristics and hematological parameters before and after therapeutic leukapheresis in order to evaluate its effect on bleeding tendency and to provide a guideline for treatment strategy. METHODS: The clinical data for 39 procedures of therapeutic leukapheresis performed on 17 patients with hyperleukocytosis from May 2005 to October 2013 at the National Cancer Center were reviewed retrospectively. RESULTS: The patients consisted of 11 males and six females. The mean age was 41 years old (range, 8~74). The mean number of therapeutic leukapheresis per patient was two (range, 1~4). Clinical symptoms improved in 14 patients (82%) after therapeutic leukapheresis and three patients (18%) were not yet to improve. The mean WBC count was significantly reduced by 32.6% (+/-17.4) after therapeutic leukapheresis, from 250,146/microL (+/-117,000) to 174,702/microL (+/-104,700) (P<0.001). The mean volume of single removal was 298 ml with 4.25x10(11)/L (+/-1.54) WBCs. After therapeutic leukapheresis, the mean platelet count showed a decline from 85x10(9)/L (+/-43) to 71x10(9)/L (+/-26). However, the prothrombin time (PT) and activated partial thromboplastin time (aPTT) did not show a significant increase (PT, P=0.637; aPTT, P=0.054). CONCLUSION: Therapeutic leukapheresis is demonstrated as an effective and safe treatment that can improve symptoms and reduce leukocytes in hyperleukocytosis.

Clinical Relevance of Hyperleukocytosis in Patients with Acute Myelogenous Leukemia / 대한진단검사의학회지

BACKGROUND: Hyperleukocytic acute myelogenous leukemia (H-AML) is a relatively rare disease found in adults and it should have different characteristics from those of non-hyperleukocytic acute myelogenous leukemia (non-H-AML). We analyzed adult patients with H-AML whose peripheral WBC count was over 100, 000/ L, and compared laboratory and clinical findings of H-AML with those of non-H-AML cases. METHODS: This study included 19 patients with H-AML who were diagnosed between July 1994 and February 2001 at Chonnam University Hospital. The laboratory data, including peripheral blood smear, bone marrow study, immunophenotyping and cytogenetic study, were reviewed and the clinical out-comes of the patients were assessed. The results were compared with those of 127 non-H-AML cases. RESULTS: Of all adult AML cases, 13.1% (19/146) were H-AML. In H-AML, the subtypes were in the order of M5 (36.8%), M4 (21.1%) and M2 (21.1%), while in non-H-AML were in the order of M2 (40.9%), M3 (28.3%) and M4 (11.0%), respectively. HLA-DR and CD14 were more frequent in H-AML than in non-H-AML (83.3% vs. 47.2%, P=0.005; and 23.5% vs. 56.4%, P=0.042; respectively). H-AML had a tendency for low complete remission and short overall survival. Disease-free survival of H-AML was significantly shorter than that for the non-H-AML (6.0 vs 22.1 months, P=0.006). CONCLUSIONS: It suggests that hyperleukocytosis could be a predictor of unfavorable clinical out-comes and survival in acute myelogenous leukemia.

A Case of Acute Sensorineural Hearing Loss as an Initial Manifestation in a Child with Acute Lymphoblastic Leukemia / 대한소아혈액종양학회지

Acute sensorineural hearing loss is unusual as initial manifestations in a child with acute lymphoblastic leukemia (ALL), even though facial or oculomotor nerve palsy as early finding of leukemia was reported. The pathology of sensorineural hearing loss in leukemia showed as leukemic cell infiltration, hemorrhage, infection, and local ischemia due to hyperviscosity. A 12-year-old boy with ALL was admitted due to multiple cervical lymphadenopathy with left sided sensorineural deafness. He complained gross hematuria and purpura on whole body. His initial complete blood cell counts were hemoglobin 11.9 g/dL, hematocrit 34.3%, white blood cells 164,000/muL (segmented neutrophils 3%, lymphocytes 11%, monocytes 2%, immature blast 84%), and platelet 28,000/muL. Pure tone audiogram revealed profound sensorineural hearing loss of the left ear at all frequencies. His brain MRI showed no definite abnormal findings without hemorrhage or infarction in inner ear or temporal lobe. He received induction chemotherapy and total 4 times of transtympanic steroid injection with 1 week interval. His hearing power at complete remission was more improved than admission, but not completely recovered until 5 months. We proposed that hearing impairment might be an initial manifestation in acute leukemia with hyperleukocytosis.

Therapeutic Leukapheresis for Acute Lymphoblastic Leukemia with Severe Hyperleukocytosis in a Six-Week-Old Infant (4.7 kg) / 대한소아혈액종양학회지

We report a 4.7 kg infant who received a therapeutic leukapheresis as an immediate treatment for acute lymphoblastic leukemia with severe hyperleukocytosis. By decreasing the number of circulating white blood cells, therapeutic leukapheresis helps prevent the risks of hyperviscosity and cerebrovascular and pulmonary leukostasis. In addition, it potentially reduces metabolic and renal complications associated with rapid cell lysis when applied before chemotherapy. This six-week-old female presented with vomiting for 15 days. Initial WBC count was 1,532,800/muL. After placement of 4 french two-lumen central venous catheter in both femoral vein, the CS 3000 plus was primed with 250 mL of paternal whole blood mixed with 150 mL of normal saline. After therapeutic leukapheresis, the CBC showed WBC count of 560,000/muL. Our successful experience in performing this procedure suggests that therapeutic leukapheresis be a feasible treatment even for very young infants with hyperleukocytosis.

Cranial Irradiation in the Management of Childhood Leukemic Hyperleukocytosis / 대한방사선종양학회지

PURPOSE: Acute leukemia with hyperleukocytosis (more than 105/mm3) is at high risk of early sudden death, usually from intracerebral hemorrhage. Emergency cranial irradiation is a relatively simple approach to solve this the problem. We summarized our experience of cranial irradiation in 24 leukemic children who presented with hyperleukocytosis. METHODS AND MATERIALS: Between 1990 and 1998, 40 children with acute leukemia presenting with hyperleukocytosis were referred for emergency cranial irradiation. Among these patients, 24 children were evaluable. There were 16 boys and eight girls, their ages ranged from 2 to 13 years (median 9.5 years). The initial leukocyte counts ranged 109,910/mm3 to 501,000/mm3. Peripheral blood smear was performed in all patients and noted the morphology of the blast. Introduction of emergency cranial irradiation was determined by the leukocyte counts (more than 100,000/mm) and the existence of the blast in peripheral blood smear. All patients were treated with intravenous hydration with alkaline fluid and oral allopurinol. Cranial irradiation started on the day of diagnosis. With 2 Gy in one fraction in 4 patients, 4 Gy in two fractions in 20 patients. RESULTS: The WBC count had fallen in 19 patients (83%) and no intracerebral hemorrhage occurred after irradiation. There were five cases of early deaths. Four patients died of metabolic complications, and one patient with intracerebral hemorrhage. He died 5 hours after cranial irradiation. No patient had any immediate side effect from cranial irradiation. CONCLUSION: Our data suggest, that emergency cranial irradiation can be safely chosen and effective in childhood leukemic patients presenting with high leukocyte counts.

Chronic myelogenous leukemia in children

A case of chronic myelogenous leukemia in a 4 month old Filipino infant, whose apparent onset was at 2 months of age, is reported. The clinical course and hematological picture of this case followed the usual features of the disease as observed in infants mainly that of hepatosplenomegaly with bleeding manifestations, hyperleukocytosis with anemia and thrombocytopenia, and a relatively short course unresponsive with the usual therapeutic measures for chronic myelogenous leukemia. Terminally the patient manifested the picture of "blastic" crisis. (Summary)

Evaluation of therapeutic leukapheresis in hyperleukocytic leukemias / 대한수혈학회지

BACKGROUND: Leukemia with hyperleukocytosis is risk factor for early mortality and morbidity. Therepeutic leukapheresis has been recognized as the choice of treatment modality to prevent leukostatic complications by selective removal of abnormal leukocytes. METHODS: We analyzed the clinical and laboratory data in total of 44 therapeutic leukapheresis performed at Samsung Medical Center in 31 patients (15 males, 16 females) with hyperleukocytic leukemias from March 1, 1995 to August 31, 1998. The change of laboratory findings related to therapeutic leukapheresis as well as the correlation between preprocedural and postprocedural hematologic parameters, the degree of leukoreduction and clinical efficacy were evaluated. RESULTS: The age distribution was from 6 months to 77 years with the 35 years of mean age. The most common diagnosis of patients who were performed therapeutic leukapheresis was acute myeloblastic leukemia (15/32, 46.9%) followed by acute lymphoblastic leukemia (9/32, 28.1%), and major leukostatic symptoms were dyspnea and headache. The mean leukocyte count before leukapheresis were 167,400/microliter and the mean leukoreduction per procedure was 50,080/microliter (30.3%). The changes of hemoglobin and platelet count were not significant. The efficacies of therapeutic leukapheresis were 66.7% in acute myeloblastic leukemia, 44.4% in acute lymphoblastic leukemia and 37.5% in other leukemia patients. Patients with low initial leukocyte count and blast count or low final leukocyte count showed higher clinical improvement rate than patients without those parameters. CONCLUSION: The present study for therapeutic leukaphresis indicate that it is relatively safe and can be used to relieve leukostatic symptoms and improve clinical status in leukemic patients.