Leukemoid reaction refers to reactive and excessive leukocytosis with a white blood cell count of more than 50,000 cells/mm3 in the absence of myeloproliferative neoplasm and has usually been described in response to inflammation, severe infection, malignancies, hemorrhage, acute hemolysis, or bone marrow stimulants. In contrast, urothelial cell carcinoma is rarely associated with leukemoid reaction, with few cases reported over the past few years. Here, we report a case of an invasive Urothelial carcinoma of a 63 year old patient presenting with terminal dysuria and excessively elevated and persisting leukocytosis accompanied by rapid tumor progression and deteriorating clinical status. For urothelial cell carcinoma patients exhibiting a leukemoid reaction, removal of the inciting tumor is the definitive treatment. Though placing of urethral catheter and resection of the bladder tumor alleviated the worsening renal status and terminal dysuria on the index patient, the patient still had multiple persistent febrile episodes throughout the course, accompanied by leukocytosis with predominant mature looking neutrophils, and a left shift. Extremely persistent leukocytosis appears to be associated with poor prognosis even after surgical resection and extensive medical management.

To improve the diagnosis of atypical lymphocytes and reduce the misdiagnosis rate,we analyzed the medical records of 2 cases with cell morphology suggestive of atypical lymphocytes.One case was diagnosed with infectious mononucleosis and the other with aggressive NK cell leukemia.The purpose of this paper is to emphasize that the diagnosis of atypical lymphocytes based only on morphological interpretation of cells may be incorrect,which should be combined with clinical symptoms,signs,imaging examination,cell immunophenotype,and disease outcome.
Humans ; Lymphocytosis/diagnosis* ; Infectious Mononucleosis/diagnosis* ; Immunophenotyping ; Diagnosis, Differential ; Diagnostic Errors

Humans ; Infectious Mononucleosis/complications* ; Lymphocytosis ; Lymphohistiocytosis, Hemophagocytic/etiology*

PURPOSE: Though prompt diagnosis to minimize symptom duration (SD) is highly associated with organ salvage in cases of testicular torsion (TT), SD is subjective and hard to determine. We thus investigated the clinical implications of systemic inflammatory responses (SIRs) as potential surrogates of SD to improve testis survival. MATERIALS AND METHODS: Sixty men with TT that underwent immediate operation among orchiectomy and orchiopexy following a visit to a single emergency department were retrospectively enrolled. Mandatory laboratory tests conducted included neutrophil, lymphocyte, and platelet counts. RESULTS: Mean age and SD was 15.7±3.7 years and 8.27±4.98 hours, respectively. Thirty-eight (63.3%) underwent orchiectomy and the remaining 22 underwent orchiopexy. Leukocytosis (p=0.001) and neutrophil-lymphocyte ratio (NLR, p < 0.001) were significantly lower in the orchiopexy group as was SD (3.27±1.88 vs. 11.16±3.80, p < 0.001). Although multivariate model showed that the only single variable associated with receipt of orchiopexy was SD (odds ratio [OR]=0.259, p < 0.001), it also revealed NLR as a sole SIR associated with SD (B=0.894, p < 0.001). While 93.3% with a SD of within 3 hours underwent orchiopexy, only 26.6% of affected testes were preserved between 3 to 12 hours (n=30). When multivariable analysis was applied to those with window period, NLR alone predicted orchiopexy rather than orchiectomy (p=0.034, OR=0.635, p=0.013). The area under curve between SD (0.882) and NLR (0.756) was similar (p=0.14). CONCLUSIONS: This study showed NLR independently predicted testis survival by proper surgical correction particularly for patients with marginally delayed diagnosis, which suggest the clinical usefulness for identifying candidates for orchiopexy in emergency setting.
Area Under Curve ; Delayed Diagnosis ; Diagnosis ; Emergencies ; Emergency Service, Hospital ; Humans ; Inflammation ; Leukocytosis ; Lymphocytes ; Male ; Neutrophils ; Orchiectomy ; Orchiopexy ; Platelet Count ; Retrospective Studies ; Spermatic Cord Torsion* ; Symptom Assessment ; Testis

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

Chronic active Epstein-Barr virus (CAEBV) infection is characterized by recurrent infectious mononucleosis (IM)-like symptoms and an unusual pattern of anti-EBV antibodies. We report a boy with CAEBV who progressed to aggressive hemophagocytic lymphohistiocytosis (HLH) with NK cell neoplasm. A 19-year-old adolescent boy was admitted with fever and a history of recurrent IM-like symptoms following mosquito bites since the age of 6 years. His condition was diagnosed as CAEBV with atypical lymphocytosis and an unusual pattern of anti-EBV antibodies. His symptoms subsided during treatment with steroids and cyclosporine, although the EBV genome load kept increasing for several years. He was re-admitted after follow-up loss for 8 years, and his clinical and laboratory findings confirmed HLH and high titer of the EBV genome. Bone marrow analysis with flow cytometry showed hemophagocytosis with compatible NK cell neoplasm. He rapidly progressed to pulmonary infection and expired soon after. We conclude that hematopoietic stem cell transplantation may be a potential therapeutic modality for treating CAEBV before serious EBV manifestations.
Adolescent ; Antibodies ; Bone Marrow ; Culicidae ; Cyclosporine ; Epstein-Barr Virus Infections ; Fever ; Flow Cytometry ; Follow-Up Studies ; Genome ; Hematopoietic Stem Cell Transplantation ; Herpesvirus 4, Human ; Humans ; Hypersensitivity ; Infectious Mononucleosis ; Killer Cells, Natural ; Lymphocytosis ; Lymphohistiocytosis, Hemophagocytic ; Male ; Steroids ; Young Adult

BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare, but potentially life threatening neurological condition in children. This study aimed to investigate its clinical spectrum, diagnostic and therapeutic dilemma, and prognosis. METHODS: Twelve children with ANE were included in the study. The diagnosis was made by clinical and radiological characteristics from January 1999 to December 2017 and their clinical data were retrospectively analyzed. RESULTS: A total of 12 children aged 6 to 93 months at onset (5 male: 7 female) were evaluated. The etiology was found in 4 of them (influenza A, H1N1; coxsackie A 16; herpes simplex virus; and RANBP2 gene/mycoplasma). The most common initial presentations were seizures (67%) and altered mental status (58%). The majority of the subjects showed elevation of aspartate aminotransferase/alanine aminotransferase with normal ammonia and increased cerebrospinal fluid protein without pleocytosis. Magnetic resonance imaging revealed increased T2 signal density in bilateral thalami in all patients, but the majority of the subjects (67%) also had lesions in other areas including tegmentum and white matter. Despite the aggressive immunomodulatory treatments, the long-term outcome was variable. One child and two sisters with genetic predisposition passed away. CONCLUSION: ANE is a distinctive type of acute encephalopathy with diverse clinical spectrum. Even though the diagnostic criteria are available, they might not be watertight. In addition, treatment options are still limited. Further studies for better outcome are needed.
Ammonia ; Aspartic Acid ; Brain Diseases ; Cerebrospinal Fluid ; Child ; Diagnosis ; Genetic Predisposition to Disease ; Humans ; Leukocytosis ; Magnetic Resonance Imaging ; Male ; Prognosis ; Retrospective Studies ; Seizures ; Siblings ; Simplexvirus ; White Matter

BACKGROUND: Spinal cord involvement of primary central nervous system lymphoma (PCNSL) is rare in a young immunocompetent patient and can be misdiagnosed as an inflammatory demyelinating disease (IDD) of the central nervous system.
Biopsy ; Brain ; Central Nervous System ; Cerebrospinal Fluid ; Cervical Cord ; Demyelinating Diseases ; Drug Therapy ; Electrons ; Hand ; Humans ; Leukocytosis ; Lymphoma ; Magnetic Resonance Imaging ; Medulla Oblongata ; Methotrexate ; Middle Aged ; Multiple Sclerosis ; Neuromyelitis Optica ; Spinal Cord

Hyperleukocytosis (HL), defined by a peripheral white blood cell (WBC) count exceeding 100,000/mm³, is occasionally observed in childhood acute leukemia. The increased viscosity in the micro-circulation by HL and the interaction between the leukemic blasts and endometrium of blood vessels sometimes result in leukostasis. Leukostasis can incur life-threatening manifestations, such as respiratory distress, brain infarction and hemorrhage, and renal failure, needing an emergency care. Although early stage of leukostasis is difficult to detect due to nonspecific manifestations, an emergency care is mandatory because leukostasis can proceed to a fatal course. Initial management includes an aggressive fluid therapy that can reduce WBC count, and prevent other metabolic complications implicated by HL. Packed red blood cells should be judiciously transfused because it increases blood viscosity. Conversely, transfusion of platelet concentrates or fresh frozen plasma, which does not affect blood viscosity, is recommended for prevention of hemorrhage. To reduce tumor burden, leukapheresis or exchange transfusion is commonly performed. However, the efficacy is still controversial, and technical problems are present. Leukapheresis or exchange transfusion is recommended if WBC count is 200,000–300,000/mm³ or more, especially in acute myelocytic leukemia, or manifestations of leukostasis are present. In addition, early chemotherapy is the definite treatment of leukostasis.
Blood Platelets ; Blood Vessels ; Blood Viscosity ; Brain Infarction ; Disease Management ; Drug Therapy ; Emergencies ; Emergency Medical Services ; Emergency Service, Hospital ; Endometrium ; Erythrocytes ; Female ; Fluid Therapy ; Hemorrhage ; Leukapheresis ; Leukemia ; Leukemia, Myeloid, Acute ; Leukocyte Disorders ; Leukocytes ; Leukocytosis ; Leukostasis ; Plasma ; Renal Insufficiency ; Tumor Burden ; Viscosity

OBJECTIVE: This study was designed to evaluate hematological disorders and the orchestrating roles of hepcidin and IL-6 in rat models of thioacetamide (TAA) and carbon tetrachloride (CCl4) hepatotoxicity. METHODS: Rats were intraperitoneally injected with TAA (10 mg/100 g rat weight dissolved in isosaline) or CCl4 (100 μL/100 g rat weight diluted as 1:4 in corn oil) twice weekly for eight consecutive weeks to induce subchronic liver fibrosis. Blood and tissue samples were collected and analyzed. RESULTS: CCl4 but not TAA significantly decreased the RBCs, Hb, PCV, and MCV values with minimal alterations in other erythrocytic indices. Both hepatotoxins showed leukocytosis, granulocytosis, and thrombocytopenia. By the end of the experiment, the erythropoietin level increased in the CCl4 model. The serum iron, UIBC, TIBC, transferrin saturation%, and serum transferrin concentration values significantly decreased, whereas that of ferritin increased in the CCl4 model. TAA increased the iron parameters toward iron overload. RT-PCR analysis revealed increased expression of hepatic hepcidin and IL-6 mRNAs in the CCl4 model and suppressed hepcidin expression without significant effect on IL-6 in the TAA model. CONCLUSION These data suggest differences driven by hepcidin and IL-6 expression between CCl4 and TAA liver fibrosis models and are of clinical importance for diagnosis and therapeutics of liver diseases.
Animals ; Blood Chemical Analysis ; Carbon Tetrachloride ; toxicity ; Hepcidins ; pharmacology ; Injections, Intraperitoneal ; Interleukin-6 ; pharmacology ; Iron ; blood ; metabolism ; Leukocytosis ; chemically induced ; therapy ; Liver Cirrhosis ; chemically induced ; therapy ; Male ; Rats ; Thioacetamide ; toxicity ; Thrombocytopenia ; chemically induced ; therapy ; Transferrin ; metabolism