Neutrophilic leukemoid reaction may occur in many situations, including hemolysis, malignancy, infection, and exposure to certain toxins. It usually shows morphological overlap with chronic myeloid leukemia in which promyelocytes are not majorly associated. Here, we present a case of promyelocytic leukemoid reaction in a patient with sepsis. A 28-year-old man was admitted for renal stone removal. After percutaneous nephrolithotomy, his condition deteriorated with fever (37.8℃), tachycardia (130/min), acute renal failure, pleural effusion, and pulmonary edema. Complete blood count indicated a white blood cell count of 73.39×10⁹/L including 82% promyelocytes, hemoglobin 8.9 g/dL, and platelet count of 85×10⁹/L. A bone marrow aspirate showed that promyelocytes accounted for 73.8% of all nucleated cells. Following bone marrow examination, treatment with all-trans retinoic acid (ATRA) was started immediately. Reverse transcription polymerase chain reaction (RT-PCR) study revealed the absence of PML-RARA (promyelocytic leukemia-retinoic acid receptor alpha) and other RARA (retinoic acid receptor alpha) rearrangements. Once the chromosome analysis of bone marrow cells demonstrated the normal karyotype, ATRA was discontinued.
Acute Kidney Injury ; Adult ; Blood Cell Count ; Bone Marrow ; Bone Marrow Cells ; Bone Marrow Examination ; Fever ; Granulocyte Precursor Cells ; Hemolysis ; Humans ; Karyotype ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Promyelocytic, Acute ; Leukemoid Reaction* ; Leukocyte Count ; Nephrostomy, Percutaneous ; Neutrophils ; Platelet Count ; Pleural Effusion ; Polymerase Chain Reaction ; Pulmonary Edema ; Reverse Transcription ; Sepsis* ; Tachycardia ; Tretinoin

Although neutrophilia can manifest from various causes, it is important to be able to distinguish chronic neutrophilic leukemia (CNL) from neutrophilic leukemoid reactions (NLR). In this paper, we describe four cases of leukocytosis with neutrophilia, including one case of CNL with a T618I mutation in colony stimulating factor 3 receptor (CSF3R) and three cases of NLR associated with malignancy or sepsis, which were initially suspected as CNL. Of the three NLR cases, one was associated with ovarian cancer, one with monoclonal gammopathy of undetermined significance and one with multiple myeloma with sepsis. This study demonstrated that confirming the clonality of myeloid cells with CSF3R T618I could contribute to making an accurate differential diagnosis between CNL and NLR in patients with solid cancers or plasma cell neoplasms caused by paraneoplastic syndromes and/or infection.
Colony-Stimulating Factors ; Diagnosis, Differential ; Humans ; Leukemia, Neutrophilic, Chronic* ; Leukemoid Reaction* ; Leukocytosis ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Myeloid Cells ; Neoplasms, Plasma Cell ; Neutrophils* ; Ovarian Neoplasms ; Paraneoplastic Syndromes ; Sepsis

Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm characterized by sustained neutrophilia, splenomegaly, and hypercellular bone marrow without Philadelphia chromosome. Diagnosis of CNL requires exclusion of identifiable causes of reactive neutrophilia, such as infection and tumors. Our patient presented with general weakness and weight loss. Computed tomography (CT) showed a mass in the distal rectum, which was confirmed to be an adenocarcinoma by colonoscopic biopsy. Positron emission tomography-CT showed multiple liver, bone, and lymph node metastases. Liver and lymph node biopsies revealed neutrophilic infiltration with no evidence of adenocarcinoma. The pathological findings of the bone marrow were compatible with CNL. Cytogenetic analysis revealed a normal karyotype, and molecular analysis was negative for BCR/ABL. Here, we present a 73 year-old man diagnosed with concurrent CNL and rectal cancer.
Adenocarcinoma ; Biopsy ; Bone Marrow ; Cytogenetic Analysis ; Diagnosis ; Electrons ; Humans ; Karyotype ; Leukemia ; Leukemia, Neutrophilic, Chronic* ; Leukemoid Reaction ; Leukocytosis ; Liver ; Lymph Nodes ; Neoplasm Metastasis ; Neutrophils ; Philadelphia Chromosome ; Rectal Neoplasms* ; Rectum ; Splenomegaly ; Weight Loss

INTRODUCTIONChildren with Down syndrome (DS) are at increased risk of developing distinctive clonal myeloid disorders, including transient abnormal myelopoiesis (TAM) and myeloid leukaemia of DS (ML-DS). TAM connotes a spontaneously resolving congenital myeloproliferative state observed in 10%-20% of DS newborns. Following varying intervals of apparent remission, a proportion of children with TAM progress to develop ML-DS in early childhood. Therefore, TAM and ML-DS represent a biological continuum. Both disorders are characterised by recurring truncating somatic mutations of the GATA1 gene, which are considered key pathogenetic events.

METHODSWe herein report, to our knowledge, the first observation on the frequency and nature of GATA1 gene mutations in a cohort of Malaysian children with DS-associated TAM (n = 9) and ML-DS (n = 24) encountered successively over a period of five years at a national referral centre.

RESULTSOf the 29 patients who underwent GATA1 analysis, GATA1 mutations were observed in 15 (51.7%) patients, including 6 (75.0%) out of 8 patients with TAM, and 9 (42.9%) of 21 patients with ML-DS. All identified mutations were located in exon 2 and the majority were sequence-terminating insertions or deletions (66.7%), including several hitherto unreported mutations (12 out of 15).

CONCLUSIONThe low frequency of GATA1 mutations in ML-DS patients is unusual and potentially indicates distinctive genomic events in our patient cohort.

Cohort Studies ; Down Syndrome ; complications ; genetics ; Exons ; Female ; GATA1 Transcription Factor ; genetics ; Gene Deletion ; Genomics ; Humans ; Infant, Newborn ; Leukemia, Myeloid ; complications ; genetics ; Leukemoid Reaction ; complications ; genetics ; Malaysia ; Male ; Mutation ; Referral and Consultation ; Remission Induction

A leukemoid reaction is usually associated with malignancies of the lung, stomach, and thyroid. In contrast, urothelial cell carcinoma is rarely associated with leukemoid reactions, with few cases reported over the past 30 years. Here, we describe a patient with urothelial cell carcinoma who exhibited a leukemoid reaction. The patient had an elevated white blood cell count and experienced a rapid and aggressive clinical course, terminating in death. For urothelial cell carcinoma patients exhibiting a leukemoid reaction, removal of the inciting tumor is the definitive treatment. However, considering the aggressive nature of these tumors, if the patient is unsuitable for radical surgical management, palliative chemotherapy should be considered.
Drug Therapy ; Humans ; Leukemoid Reaction* ; Leukocyte Count ; Lung ; Paraneoplastic Syndromes* ; Prognosis* ; Stomach ; Thyroid Gland

A leukemoid reaction is defined as reactive leukocytosis exceeding 50,000/mm3, with a significant increase in early neutrophil precursors, and can be a paraneoplastic manifestation of various malignant tumors. A 71-year-old male patient complained of decreased appetite, fatigue, and abdominal fullness. He had a palpable, firm liver, and laboratory investigations suggested leukemoid reaction. Liver dynamic computed tomography revealed a hypervascular mass, and an ultrasound-guided fine-needle aspiration of the mass confirmed hepatocellular carcinoma (HCC) with a sarcomatoid component. The leukocyte count of the patient had increased to 147,800/mm3, and he died 10 days after admission. This is a rare case of leukemoid reaction in a patient with sarcomatous HCC.
Aged ; Biopsy, Fine-Needle ; Carcinoma, Hepatocellular/*pathology ; Humans ; Leukemoid Reaction/*diagnosis ; Leukocyte Count ; Liver Neoplasms/*pathology ; Male ; Tomography, X-Ray Computed

PURPOSE:Leukemoid reaction (LR) represents inflammatory reaction in very low birth weight (VLBW) infants, and has been reported to relate with the development of bronchopulmonary dysplasia (BPD). This study was done to assess the relationship between perinatal characteristics and neonatal outcome of leukemoid reaction in VLBW infants. METHODS:Three hundred and seventy two VLBW infants admitted to the NICUs of SNUCH and SNUBH between June 2005 to June 2008 were studied in a retrospective case-controlled manner. Of these VLBW infants, 32 LR (+) infants were compared with 83 LR (-) infants who were matched for gestational age and birth weight with respect to perinatal and neonatal clinical characteristics. LR was defined as a white blood cell (WBC) count of more than 40,000 cells/mm3 or absolute neutrophil count of more than 30.000 cells/mm3 during the first month of life. RESULTS:Of 413 VLBW infants, There was no significant difference between the LR (+) and LR (-) infants in sex, Apgar scores, maternal peripheral WBC count, the use of antenatal steroids and maternal age. Comparing with LR (-) infants, LR (+) infants had a higher positive rate for amniotic fluid culture (67% vs 11%; P< or =0.01), U. ureaplasma in amniotic fluid (52% vs 10%; P=0.01), histologic chorioamnionitis (81% vs 33%; P=0.01) and funisitis (50% vs 7%; P=0.01). The incidences of respiratory distress syndrome, patent ductus arteriosus, necrotizing enterocolitis, sepsis, intraventricular hemorrhage and BPD showed no significant difference between LR (+) and LR (-) infants. CONCLUSION:LR during the first month of life in VLBW infants was associated with chorioamnionitis, but not with the incidence of bronchopulmonary dysplasia or other neonatal morbidities. These findings suggest that LR may be a simple reflection of transient accelerated neutrophil production induced by prenatal inflammation without significant neonatal consequences.
Amniotic Fluid ; Birth Weight ; Bronchopulmonary Dysplasia ; Case-Control Studies ; Chorioamnionitis ; Ductus Arteriosus, Patent ; Enterocolitis, Necrotizing ; Female ; Gestational Age ; Hemorrhage ; Humans ; Incidence ; Infant ; Infant, Newborn ; Infant, Very Low Birth Weight ; Inflammation ; Leukemoid Reaction ; Leukocytes ; Maternal Age ; Neutrophils ; Polymethacrylic Acids ; Pregnancy ; Retrospective Studies ; Sepsis ; Steroids ; Ureaplasma

PURPOSE: This study determined the prenatal and postnatal factors associated with complications and prognosis in premature infants with leukemoid reaction. METHODS: We retrospectively reviewed the medical records of premature infants with gestational ages <37 weeks and low birth weights (<2,500 g) who were admitted immediately after birth to the neonatal intensive care unit at the Dongguk University Ilsan Hospital between June 2005 and July 2006. A leukemoid reaction was defined as an absolute neutrophil count (ANC) >30,000/mm3. The infants who had leukemoid reaction comprised the study group, while the remainder of infants made up the control group. The relationships between maternal and neonatal variables and ANC were studied. RESULTS: Leukemoid reaction was detected in 3.1% of the study infants (8 of 252). Factors more frequently associated with infants with leukemoid reaction were as follows: maternal chorioamnionitis, high levels of maternal and infant C-reactive protein, gestational age <37 weeks, birth weight <2,500 g, low Apgar score, prolonged ventilator support, and a high incidence of bronchopulmonary dysplasia (BPD). However, there were no significant differences with respect to the antenatal usage of steroids, the incidences of patent ductus arteriosus, necrotizing enterocolitis, intraventricular hemorrhage, retinopathy of prematurity, and mortality between the two groups. CONCLUSION: Leukemoid reaction in premature infants was associated with chorioamnionitis and high levels of serum C-reactive protein in mothers and infants, and BPD in infants. These findings suggest that leukemoid reaction is secondary to inflammation caused by infection.
Apgar Score ; Birth Weight ; Bronchopulmonary Dysplasia ; C-Reactive Protein ; Chorioamnionitis ; Ductus Arteriosus, Patent ; Enterocolitis, Necrotizing ; Female ; Gestational Age ; Hemorrhage ; Humans ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Inflammation ; Intensive Care, Neonatal ; Leukemoid Reaction ; Medical Records ; Mothers ; Neutrophils ; Parturition ; Pregnancy ; Prognosis ; Retinopathy of Prematurity ; Retrospective Studies ; Steroids ; Ventilators, Mechanical

Pseudomembranous colitis (PMC) is highly prevalent in patients with broad spectrum antibiotic therapy. It can result in significant morbidity and mortality, especially if it is not diagnosed early. The clinical manifestation of PMC is diverse and symptoms usually are increased order of severity. Although leukocytosis is common, leukemoid reaction is very rare in PMC. We report a case of PMC associated with a leukemoid reaction without typical symptoms in a type 2 diabetic patient who have the multiple diabetic complications.
Clostridium difficile ; Diabetes Complications ; Diabetes Mellitus ; Enterocolitis, Pseudomembranous* ; Humans ; Leukemoid Reaction* ; Leukocytosis ; Mortality

Blood Cells ; immunology ; pathology ; Bone Marrow Cells ; immunology ; pathology ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Immunophenotyping ; Leukemoid Reaction ; diagnosis