Long-term outcome for Down syndrome patients with hematopoietic disorders.

BACKGROUND/PURPOSE: Although Down syndrome (DS) patients have a higher risk of developing transient myeloproliferative disorder (TMD) and acute leukemia, very little data is available on long-term outcome in Taiwanese patients. The current study was designed to determine the clinical characteristics and treatment outcome of DS patients with TMD or acute leukemia (AL). METHODS: In 25 consecutive DS patients with TMD or AL enrolled from 1990 to 2012, clinical manifestations and treatment protocols were investigated and GATA1 (GATA binding protein 1) mutations were identified. Among 16 DS-acute myeloid leukemia (DS-AML) patients, clinical outcomes were compared between survivors and nonsurvivors. RESULTS: Most of our DS patients had TMD (32%), acute megakaryoblastic leukemia (24%), or acute erythromegakaryoblastic leukemia (16%). The median follow-up time was 22.5 months (1-230 months). The age was younger and the hemoglobin (Hb) level and platelet count were higher in TMD patients than in leukemia patients. Among DS-AML patients, the Hb level was higher in survivors than nonsurvivors (8.8 ± 2.7 g/dL vs. 5.8 ± 2.4 g/dL; p = 0.044) and the age was older in relapsed patients than in nonrelapsed patients (43.8 ± 18 months old vs. 21.6 ± 8.6 months old; p = 0.025). The 3-year overall survival (OS) rate was 44%, higher in patients receiving appropriate chemotherapy than in those receiving inadequate treatment (63.6% vs. 0%, p = 0.001), and higher in those diagnosed with TMD or AL after 2008 than before 2008 (33.3% vs. 75%; p = 0.119). CONCLUSION: Outcome in DS-AML patients is optimal if appropriate treatment is provided. With modification of the treatment strategy in 2008, OS increased in Taiwan.

Retrospective analysis of leukemoid reactions in extremely preterm infants in a tertiary NICU from 2018-2021.

BACKGROUND: Neonatal leukemoid reaction is associated with higher risk of mortality, chronic lung disease and has been associated with chorioamnionitis. Literature on extremely low birth weight infants with leukemoid reaction is limited. OBJECTIVES: The aim of our study was to characterize the maternal and placental factors associated with neonatal leukemoid reaction and to describe outcomes of these ELBW infants. Our objective was to assess if there were maternal factors that would assist the decision-making process regarding the delivery of preterm infants at risk of chorioamnionitis and the sequelae of this inflammatory process. METHODS: This was a retrospective case-control study performed in a single, tertiary Maternity Hospital in Dublin. Two matched controls were identified for each case based on gestation and year of birth and data was collected on both the infants and their mothers. RESULTS: 7 extremely preterm neonates were identified as having a leukemoid reaction, defined as a total white cell count of >50,000 or in the first seven days of life. Baseline characteristics between the groups were similar. The median gestational age in the cases group was 24 + 4 weeks and in the control group was 24 + 1. The mean birthweight was 650 g in the cases group vs. 655 g in the control group. There was a higher percentage of males in the control group, 42.9% vs 28.6% in the cases. The preterm infants with leukemoid reaction had a longer duration of ventilation with a median of 18 days (7.5-23.5 days) compared to 6.5 days (2.8-24.5 days) in the control group. More infants in the leukemoid reaction group required inotropes for hypotension in the first 72 h after delivery (42.9% vs 7.1% in the controls, p value .169). Death or Bronchopulmonary dysplasia (BPD) occurred in 85.7% of the cases identified with a leukemoid reaction vs 71.4% of the controls matched. Median maternal CRP was higher in cases prior to delivery vs the controls (66 vs 18.1 mg/L, p-value = .2151). There was histological evidence of maternal inflammatory response in all cases with fetal inflammatory response in 71% of cases. CONCLUSIONS: Leukemoid reaction in ELBW infants with evidence of maternal and fetal inflammatory response syndrome on placental histology is associated with a longer duration of initial ventilation, increased need for inotropes in the first 72 h after birth, higher rates of death, and BPD. Prospective studies are required to identify potential biomarkers such as proinflammatory cytokines, IL-6, which might aid the decision-making process in delivery.

Histological chorioamnionitis and neonatal leukemoid reaction in low-birth-weight infants.

We investigated whether histological chorioamnionitis (HCA) is a risk factor predisposing to leukemoid reaction (LR) and whether LR is associated with the preterm parturition syndrome and the systemic fetal inflammation response syndrome. A prospective histological study on placentas was performed in preterm infants (

Leukaemoid reaction in Eastern Highlands children.

In the five year period 1973-77, 278 children in Goroka Hospital paediatric ward were found to have a white cell count (W.C.C.) over 30,000/mm3. Of those with a W.C.C. over 40,000/mm3 in 1977 where the outcome was known, 13 of the 17 (76%) died. It is suggested that "leukaemoid reaction" in Highlands children be defined as a total W.C. over 40,000/mm3 that is not due to leukaemia. A leukaemoid reaction is a useful, but grave, prognostic sign. Such children comprise a quarter of all deaths in the Goroka paediatric ward.

The relationship between leukemoid reaction and perinatal morbidity, mortality, and chorioamnionitis in low birth weight infants.

OBJECTIVES: Neonatal leukemoid reaction (NLR) is relatively rare and considered as a white blood cell (WBC) count ≥50×10(9)/l . The aim of this study was to investigate the association of NLR with neonatal morbidity and mortality and maternal chorioamnionitis in low birth weight infants. METHODS: In this case-controlled retrospective study, the medical records of 1200 newborn infants with a birth weight <2500g admitted to the neonatal unit over a period of 5 years were reviewed. The infants who developed features of NLR (n=17, 1.4%) formed the study group, while the remainder without NLR, matched for gestational age and birth weight (n=123), formed the control group. A chart review was performed and salient demographic, clinical, and laboratory data abstracted. A statistical analysis was subsequently performed on this data. RESULTS: The mean WBC and absolute neutrophil counts of infants with NLR were significantly higher than those in the control group. The peak time of NLR was at 7.9±3.6 (interquartile range (IQR) 1-30) days and on average it improved within 4.1±1.95 (IQR 2-9) days. It was noted that those infants with NLR were mostly born by vaginal delivery and their mothers had a higher rate of early rupture of the membranes and chorioamnionitis. NLR was associated with a 4-fold increase in sepsis, 20-fold increase in intraventricular hemorrhage, 54-fold increase in bronchopulmonary dysplasia, and 6-fold increase in mortality. In the study group, those infants whose mothers had chorioamnionitis had a higher rate of early rupture of the membranes and they developed sepsis and intraventricular hemorrhage more often than those whose mothers did not have clinical chorioamnionitis. CONCLUSIONS: In low birth weight newborn infants, NLR is significantly associated with sepsis, intraventricular hemorrhage, bronchopulmonary dysplasia, and a high mortality rate. Also, those infants with NLR are more likely to be born to mothers with chorioamnionitis and they face sepsis and intraventricular hemorrhage more often.

Myeloid leukaemoid reactions in South African Blacks.

Myeloid leukaemoid reactions were observed in 18 Black adults and 20 Black children at Baragwanath Hospital, Johannesburg, during a 10-month period. This represented an incidence of 1,3/1 000 adult medical admissions and 3,7/1 000 paediatric medical admissions. During the same period the incidence of myeloid leukaemoid reactions in White children in Johannesburg and in Black and White children in Buffalo, NY, was less than 1/1 000 admissions. The reactions were associated with bacterial infections, neoplasms, hepatorenal failure, acute metabolic disorders and non-malignant blood dyscrasias. The mortality in adults was 55,6% and in children 35%. Sixty-five per cent of all deaths occurred within 24--48 hours of admission. Residual morbidity in survivors was common. The pattern of acute leukaemia in the same hospital population is predominantly myelocytic in both adults and children.

The leukemoid reaction in shigellosis.

Leukemoid reactions occurred in 136 patients (3.8%) hospitalized with shigellosis in Bangladesh. Sixty-eight percent of the patients with leukemoid reactions were children less than 4 years old. When compared with patients without leukemoid reactions, the leukemoid reactions were significantly associated with children aged less than 10 years. The most common serotype of Shigella in the patients with leukemoid reactions was Shigella dysenteriae 1, isolated from 96 patients (71%), whereas the most common species in patients without leukemoid reactions was Shigella flexneri, isolated from 2,119 patients (62%). The case fatality rate in patients with leukemoid reactions was 21% compared with 7.4% in patients without leukemoid reactions. These findings indicated that in patients with shigellosis, the leukemoid reaction was significantly associated with young children, isolation of S dysenteriae 1, and increased case fatality rate.

Relationship between neonatal leukemoid reaction and bronchopulmonary dysplasia in low-birth-weight infants: a cross-sectional study.

Leukemoid reaction in low-birth-weight (LBW) infants is a rare, recently documented phenomenon, implicated in the sequence of multiorgan inflammatory diseases of preterm infants. The aim of the present paper is to establish whether a neonatal leukemoid reaction is related to bronchopulmonary dysplasia (BPD) development in LBW infants. The design was a case-controlled, retrospective study of all premature infants (born 40,000/mm 3. The relation between BPD occurrence and WBC counts was studied by Bayesian analysis, dividing WBC counts in 5 progressive bands of 10,000 WBC/mm 3, starting from <10,000 to >40,000/mm 3. Five of 50 BPD infants studied demonstrated WBC counts >40,000/mm 3, with an incidence of 10%, while no control preterm infants presented neonatal leukemoid reaction; the estimated number difference is statistically significant ( p <0.001). There was no other significant association demonstrated between maternal or neonatal variables and leukemoid reaction, including chorioamnionitis, sepsis, and the use of antenatal steroids. Our findings provide further data for the identification of prematures exposed to pro-inflammatory cytokines in utero; the injury responsible for BPD in a subset of prematures may begin with a transient leukemoid reaction.

Shiga bacillus dysentery associated with marked leukocytosis and erythrocyte fragmentation.

Granulocytic leukemoid reactions (white blood cell counts greater than 50,000 with myelocytes and promyelocytes in the peripheral blood) were documented in 15 per cent of 273 patients with dysentery due to Shigella dysenteriae, type 1 (Shiga bacillus) in Bangladesh. Peak granulocytosis occurred during the second week of illness, when the children were commonly afebrile and diarrhea had ceased or was subsiding. More than half of the patients with leukemoid reactions subsequently developed a fall in hematocrit associated with striking erythrocyte fragmentation on blood smears. Thrombocytopenia occurred during the period of hemolysis in most. Transient oliguric renal failure developed in several patients. Most made a complete recovery. The pathogenesis of the syndrome and the reason for its high incidence were not determined.

Comparison of bone marrow histology in early chronic granulocytic leukemia and in leukemoid reaction.

A retrospective study was performed on bone marrow biopsies of 50 untreated patients with leukemoid reactions (LR) and 50 untreated patients with early chronic granulocytic leukemia (CGL). A comparison was made between hematopoietic and adipose tissues, bone and its cells, as well as other stromal components in these two disorders. Histologic and histomorphometric analyses revealed significant differences in trabecular structure, in localization of fat cells, in numbers of sinusoids, capillaries and various stromal elements. No significant differences between LR and CGL were detected in the quantity of erythro- and granulocytopoiesis and of megakaryocytes, but these were smaller in CGL than in LR. This histologic and histomorphometric evaluation demonstrates that certain histologic features may serve as valuable aids in distinguishing LR from CGL.

Leukaemoid reaction in diarrhoeal diseases.

There was an outbreak of diarrhoea/dysentery in Naxalbari, North Bengal in August-September of 1992. Ninety-seven cases were investigated. Bacterial pathogens were isolated from stools of 17 cases and the organisms were Salmonella typhimurium (76%), Salmonella enteritidis (12%) and Shigella dysenteriae type 1(12%). A leukaemoid reaction was observed in 4 cases (24%) amongst all 17 patients and they were all children.

Leukemoid reaction in extremely low-birth-weight infants.

The objective of this paper was to determine the incidence of leukemoid reaction and to evaluate its relationship with maternal and neonatal factors in extremely low-birth-weight (ELBW) infants. The design a case-controlled retrospective study of all live-born ELBW infants (<1000 g) over a period of 2 years, from July 1994 to June 1996. A total of 60 preterm infants were born during the study period, and are included in this report. The infants who demonstrated leukemoid reaction formed the study group, while the remainder formed the control group. Leukemoid reaction was defined as a white blood cell (WBC) count > or = 50,000/mm3. The relationship between maternal and neonatal variables and WBC counts was studied. Nine of the 60 infants studied demonstrated counts >50,000/mm3, with an incidence of 15%. There was no significant association demonstrated between maternal or neonatal variables and leukemoid reaction. Patients with leukemoid reaction had a better overall survival. Leukemoid reaction in ELBW infants is a rare and recently documented phenomenon. In our study the incidence was 15%. Although many factors have been postulated as a cause of this phenomenon, we could not demonstrate any relationship between these factors and high WBC counts, including sepsis and the use of antenatal steroids, the two most likely variables. It was interesting to note that the infants who were able to mount a leukemoid response had a better chance of survival than those who did not.