Clinical characteristics and CT findings of Pneumocystis Jirovecii pneumonia in 46 cases with hematological diseases / 中华血液学杂志

Objective: To summarize the original CT features of Pneumocystis Jirovecii pneumonia in patients with hematological diseases. Methods: A retrospective analysis was carried out in 46 patients with proven pneumocystis pneumonia (PJP) in the Hospital of Hematology, Chinese Academy of Medical Sciences between January 2014 and December 2021. All patients had multiple chests CT and related laboratory examinations, imaging typing were conducted based on the initial CT presentation, and the distinct imaging types were analyzed against the clinical data. Results: In the analysis, there were 46 patients with proven pathogenesis, 33 males, and 13 females, with a median age of 37.5 (2-65) years. The diagnosis was validated by bronchoalveolar lavage fluid (BALF) hexamine silver staining in 11 patients and clinically diagnosed in 35 cases. Of the 35 clinically diagnosed patients, 16 were diagnosed by alveolar lavage fluid macrogenomic sequencing (BALF-mNGS) and 19 by peripheral blood macrogenomic sequencing (PB-mNGS) . The initial chest CT presentation was categorized into 4 types, including ground glass (GGO) type in 25 cases (56.5%) , nodular type in 10 cases (21.7%) , fibrosis type in 4 cases (8.7%) , and mixed type in 5 cases (13.0%) . There was no substantial discrepancy in CT types among confirmed patients, BALF-mNGS diagnosed patients and PB-mNGS diagnosed patients (χ(2)=11.039, P=0.087) . The CT manifestations of confirmed patients and PB-mNGS diagnosed patients were primarily GGO type (67.6%, 73.7%) , while that of BALF-mNGS diagnosed patients were nodular type (37.5%) . Of the 46 patients, 63.0% (29/46) had lymphocytopenia in the peripheral blood, 25.6% (10/39) with positive serum G test, and 77.1% (27/35) with elevated serum lactate dehydrogenase (LDH) . There were no great discrepancies in the rates of lymphopenia in peripheral blood, positive G-test, and increased LDH among different CT types (all P>0.05) . Conclusion: The initial chest CT findings of PJP in patients with hematological diseases were relatively prevalent with multiple GGO in both lungs. Nodular and fibrosis types were also the initial imaging findings for PJP.

Retrospective Analysis of Irregular Antibodies Causing Hemolytic Disease of the Fetus and Newborn in Jiangxi Province / 中国实验血液学杂志

OBJECTIVE@#To analyze the characteristics of antibody-specific distribution, laboratory detection results of hemolytic disease of the fetus and neonatal(HDFN) caused by irregular blood group antibodies other than ABO, and its correlation with the clinical situation.@*METHODS@#The non-ABO-HDFN cases in our hospital from October 2012 to December 2021 were selected as the research objects, and the cases diagnosed with ABO-HDFN in the same period were randomly selected as the control group, and the data of antibody specific distribution, total bilirubin, direct antibodies, maternal history, age of the children, the presence or absence of combined ABO-HDFN, and whether to exchange/transfuse blood were retrospectively analyzed. The characteristics of non-ABO-HDFN in Jiangxi province were analyzed.@*RESULTS@#The detection rate of non-ABO-HDFN in Jiangxi province increased. Among 187 non ABO-HDFN cases, the highest percentage of Rh-HDFN was detected (94.6%). Compared with the control group of ABO-HDFN, the non-ABO-HDFN had higher mean integral value of direct antibody, higher peak total bilirubin, and longer duration. Anti-M-HDFN may have severe disease but the direct antibody weak positive/negative, it was easy missed in clinical and delayed the treatment. There is no correlation between the specificity of irregular antibodies, the sex of the child, the mother's previous childbirth history, the presence or absence of combined ABO-HDFN and the need for blood exchange/transfusion(P>0.05).@*CONCLUSION@#The irregular antibodies of causing non ABO-HDFN in Jiangxi area are mainly Rh blood group system, followed by MNS blood group system. Understanding the characteristics of HDFN disease, serological features and the correlation with clinical indexes will help to detect and treat non ABO-HDFN in time and reduce the risk of complications.

Analysis of Pathogenic Bacterial Spectrum, Drug Resistance and Risk Factors for Mortality of Bloodstream Infection in Patients with Hematologic Diseases / 中国实验血液学杂志

OBJECTIVE@#To analyze the pathogenic bacterial spectrum, drug resistance, and risk factors associated with multidrug-resistant bacterial infection and mortality in patients with hematologic diseases complicated by bloodstream infections, so as to provide reference for rational drug use and improving prognosis.@*METHODS@#Positive blood culture specimens of patients with hematologic diseases in two Class A tertiary hospitals of Shanxi province from January 2019 to December 2021 were retrospectively analyzed. Pathogen distribution, drug resistance and outcomes of patients with bloodstream infection were investigated, then the multivariate logistic analysis was performed to analyze the risk factors of multidrug-resistant bacterial infection and factors affecting prognosis.@*RESULTS@#203 strains of pathogens were identified, mainly Gram-negative bacteria (GNB) (69.46%, 141/203), of which Escherichia coli (E.coli) had the highest incidence (41.13%, 58/141), followed by Klebsiella pneumoniae (20.57%, 29/141) and Pseudomonas aeruginosa (12.77%, 18/141). Extended-spectrum beta-lactamase (ESBL)-producing E.coli and Klebsiella pneumoniae were 46.55% (27/58) and 37.93% (11/29), respectively. Carbapenem-resistant Gram-negative bacteria accounted for 10.64% (15/141). And Gram-positive bacteria accounted for 27.59% (56/203), Staphylococcus epidermidis, Streptococcus pneumoniae, and Staphylococcus aureus were the most frequently isolated pathogen among Gram-positive bacteria (14.29%, 12.50% and 10.71%, respectively), of which methicillin-resistant Staphylococcus aureus accounted for 33.33% (2/6), coagulase-negative staphylococci accounted for 87.50% (7/8), without vancomycin- or linezolid-resistant strain. Additionally, fungi accounted for 2.95% (6/203), all of which were Candida. Multidrug-resistant Gram-negative bacteria (MDR-GNB) accounted for 53.90% (76/141). Duration of neutropenia >14 days was a risk factor for developing MDR-GNB infection. The 30-day all-cause mortality was 10.84%. Multivariate logistic regression analysis showed that the significant independent risk factors for mortality were age≥60 years (P <0.01, OR =5.85, 95% CI: 1.80-19.07) and use of vasopressor drugs (P <0.01, OR =5.89, 95% CI: 1.83-18.94).@*CONCLUSION@#The pathogenic bacteria of bloodstream infection in patients with hematological diseases are widely distributed, and the detection rate of multidrug-resistant bacteria is high. The clinicians should choose suitable antibiotics according to the results of bacterial culture and antibiotic susceptibility test.

Incidence and clinical characteristics of engraftment syndrome after syngeneic hematopoietic stem cell transplantation in patients with hematological diseases / 中华血液学杂志

Objective: To explore the incidence and clinical characteristics of engraftment syndrome (ES) after syngeneic hematopoietic stem cell transplantation (syn-HSCT) in patients with hematological diseases. Methods: The clinical data of 21 patients who received syn-HSCT at People's Hospital of Peking University from January 1994 to May 2018 were retrospectively analyzed. Results: Seven (33.3% ) of 21 patients developed ES. The onset of ES symptoms occurred at a median of 8 (range: 5-13) days after HSCT, and the diagnosis of ES occurred at a median of 10 (range: 7-14) days after HSCT. Steroids were administered immediately after the diagnosis of ES, the median time of symptom continuance was 2 (range: 1-5) days, and all patients showed complete resolution of ES symptoms. In the multivariate analysis, patients with acute myeloid leukemia and faster neutrophil reconstitution were the risk factors for ES (HR=15.298, 95% CI 1.486-157.501, P=0.022, and HR=17.459, 95% CI 1.776-171.687, P=0.014) . Meanwhile, there was no significant difference in the overall survival and disease-free survival between patients with ES and those without ES. Conclusion: A high incidence of ES was observed in syn-HSCT recipients. Moreover, the prognosis of ES was excellent.

Correlation between Serum Homocysteine Level, MTHFR Gene Polymorphism and Patients with Hematological Diseases Complicated with Coronary Heart Disease / 中国实验血液学杂志

OBJECTIVE@#To observe the expression level of serum homocysteine (Hcy) and methylenetetrahydrofolate reductase (MTHFR) gene polymorphism in patients with hematological diseases complicated with coronary heart disease, and analyze the relationship between serum Hcy level, MTHFR gene polymorphism and coronary heart disease.@*METHODS@#The medical records of 80 patients with coronary heart disease who completed treatment of hematological diseases during the period from March 2018 to March 2020 were selected as observation group. In addition, the medical records of 92 patients with hematological diseases who completed treatment in our hospital during the same period were selected as control group. Venous blood samples of the two groups were collected to detect serum Hcy level and MTHFR gene polymorphism. The serum Hcy levels of the two groups with different MTHFR genotypes were compared, and the effects of the above indicators on hematological diseases complicated with coronary heart disease were analyzed.@*RESULTS@#The detection rates of MTHFR gene TT and TC in the observation group were higher than those in the control group, while the distribution frequency of MTHFR genotype CC was lower (P<0.05). The serum Hcy levels of the patients with MTHFR genotype TT and TC in the observation group was higher than the control group (P<0.05). Binary logistic regression analysis showed that MTHFR gene TC/CC genotype serum Hcy overexpression may be influencing factor which induced coronary heart disease in patients with hematological diseases (OR=2.107/OR=1.634, P<0.05). ROC curves showed that the AUC of serum Hcy level of MTHFR gene TC/CC genotype and hematological disease complicated with coronary heart disease were both > 0.8. When MTHFR gene TC reaching the optimal threshold of 22.165 μmol/L, the sensitivity was 0.950 and the specificity was 0.837, While MTHFR gene CC reached the optimal threshold of 19.630 μmol/L, the sensitivity was 0.938 and the specificity was 0.826, the best predictive value could be obtained.@*CONCLUSION@#The changes of serum Hcy and MTHFR gene polymorphisms may be involved in the pathological process in patients with hematological diseases complicated with coronary heart disease. In the future, early detection of serum Hcy levels and MTHFR gene polymorphisms in patients with hematological diseases can be used to predict the risk of coronary heart disease.

Lípidos anormales y síndrome de inmunodeficiencia adquirida: asociación de lípidos séricos con el estado del VIH / Abnormal Lipids and Acquired Immune Deficiency Syndrome: Association of Serum Lipids with HIV Status

Contexto y objetivo: las anomalías hematológicas se encuentran entre las complicaciones más comunes de la infección por el VIH. También se han realizado bastantes estudios sobre las alteraciones en el perfil de lípidos, aunque los resultados en gran medida no han sido concluyentes. El presente estudio se llevó a cabo para evaluar el recuento de células CD4 y el perfil de lípidos en los pacientes infectados por el VIH y el SIDA en la población india y los correlaciona con los controles sero-negativos. Materiales y métodos: El presente estudio fue diseñado como un estudio transversal, con base en un hospital, para evaluar el recuento de células CD4 y el perfil de lípidos en los pacientes infectados por VIH y SIDA en la población india y los correlaciona con los controles sero-negativos. La evaluación del perfil de lípidos se realizó utilizando Erba EM 360, un analizador automático impulsado por un fotómetro de rejilla de difracción, mientras que los recuentos de células CD4 se evaluaron utilizando el Contador de ciclo de Partec. Análisis estadístico utilizado: Los datos se analizaron con SPSS versión 15.0 (SPSS Inc., Chicago, IL, EE. UU.). La comparación de dichos parámetros se realizó mediante el análisis de varianza (ANOVA) y la prueba post-hoc de Games-Howell. Se consideró estadísticamente significativo un valor de p <0,05. Resultados: Los niveles de colesterol total y lipoproteínas de baja densidad (LDL) disminuyeron significativamente, mientras que los triglicéridos y las lipoproteínas de muy baja densidad (VLDL) aumentaron significativamente en los pacientes infectados por VIH y SIDA en comparación con los controles sero-negativos. Conclusión: El colesterol total, las LDL, los triglicéridos y las VLDL se alteraron significativamente en los pacientes infectados por VIH y con SIDA en comparación con los controles sero-negativos

Context and Aim: Hematological abnormalities are amongst the most common complications of infection with HIV.There have been quite a few studies on the alterations in lipid profile, too, though the results have largely been inconclusive. The present study was carried-out to assess CD4 cell counts and lipid profile in the HIV infected and AIDS patients in the Indian population and correlates them with the sero-negative controls. Materials and Methods: The present study was designed as a cross-sectional, hospital-based study to assess CD4 cell counts and lipid profile in the HIV infected and AIDS patients in the Indian population and correlates them with the sero-negative controls. Evaluation of lipid profile was done using Erba EM 360, an automated analyzer powered by a diffraction grating photometer while CD4 cell counts were evaluated using Partec Cyflow Counter. Statistical analysis used: The data was analyzed using SPSS version 15.0 (SPSS Inc., Chicago, IL, USA). Comparison of the said parameters was done using Analysis of Variance (ANOVA) and post-hoc Games-Howell test. p-value of <0.05 was considered statistically significant. Results: The levels of total cholesterol and low-density lipoproteins (LDLs) were significantly decreased while triglycerides and very low density lipoproteins (VLDLs) were significantly increased in the HIV infected and AIDS patients when compared with the sero-negative controls. Conclusion: Total cholesterol, LDLs, triglycerides and VLDLs were significantly altered in the HIV infected and AIDS patients when compared with the sero-negative controls

Clinical Features and Prognosis of Patients with Hematological Disease Complicated with Mucor Infection / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical features, treatment and prognosis of patients with hematological diseases complicated with mucor infection.@*METHODS@#The risk factors, clinical features, treatment regimen and prognosis of 18 hematological disease patients with mucor infection diagnosed by histopathology in our center from April 2014 to June 2020 were retrospectively analyzed.@*RESULTS@#Thirteen males and five females, with an average age of 30 (13－54) years old, were diagnosed as mucor infection by histopathological examination at the site of infection, including 16 cases of mucor infection alone and 2 cases of mucor + aspergillus mixed infection. There were 12 cases with malignant hematological disease and 6 cases with severe aplastic anemia, all of whom with long-term agranulocytosis, and their clinical manifestations and imaging findings were not specific. The common sites of infection were sinuses and lungs, and some patients showed multiple systemic manifestations. The remission status of hematological diseases and recovery of immune function showed an impact on the prognosis. All the patients were treated with amphotericin B liposome combined with posaconazole, and 15 patients were treated with surgery combined with antifungal drugs, 9 of whom were effective and 6 were ineffective, while intravenous administration in 3 cases was ineffective.@*CONCLUSION@#It is difficult to diagnose hematological disease complicated with mucor infection. After early diagnosis, prognosis can be improved by amelioration of primary state and combination of drugs and surgery.

Hipervitaminosis B12: Nuestra experiencia y una revisión / Hypervitaminosis B12. Our experience and a review

Los altos niveles de vitamina B12 o cobalamina, también denominado hipervitaminosis B12 es una anormalidad analítica frecuentemente subestimada. De acuerdo con la literatura algunas de las entidades relacionadas con este hallazgo son las neoplasias sólidas (primarias o metastásicas) y las enfermedades hematológicas agudas o crónicas. Otras causas incluyen la afección hepática, la gammapatía monoclonal de significación indeterminada, la insuficiencia renal y, con menor frecuencia, un exceso de consumo de vitamina B12, enfermedades inflamatorias o autoinmunes y los trastornos hematológicos transitorios (neutrofilia y eosinofilia secundaria). Este artículo informa sobre causas de hipervitaminosis B12, nuestra experiencia y hace una revisión de la literatura.

High serum levels of vitamin B12 or cobalamin, also called hypervitaminemia B12, is a frequently underestimated biological abnormality. According to the literature, some of the entities related to this finding are solid neoplasia (primary or metastatic) and acute or chronic hematological diseases. Other causes include liver disorders, monoclonal gammapathy of undetermined significance, renal failure and, less frequently, excess of vitamin B12 intake, inflammatory or autoimmune diseases, and transient hematological disorders (neutrophilia and secondary eosinophilia). This article reports on causes of hypervitaminosis B12, our experience and a review of the literature.

Características clínico microbiológicas de la infección por Acinetobacter baumannii en pacientes con afecciones hematológicas / Clinical microbiological characteristics of Acinetobacter baumannii infection in patients with hematological disorders

Introducción: Las complicaciones infecciosas se han convertido en la causa de mortalidad más frecuente en los pacientes con afecciones hematológicas sometidos a regímenes terapéuticos agresivos. Objetivo: Caracterizar la infección por Acinetobacter baumannii en pacientes con afecciones hematológicas. Métodos: Se realizó un estudio ambispectivo y descriptivo en el servicio de hematología del Hospital Clínico Quirúrgico Hermanos Ameijeiras, entre enero de 2010 y diciembre de 2016. La muestra se conformó con 29 pacientes que cumplieron los criterios de inclusión establecidos. Resultados: La edad promedio fue de 48,3±14,8 años, con predominio del sexo masculino (65,5 por ciento). Resultaron más frecuentes los casos con linfomas (48,3 por ciento). En cuanto al estado de la enfermedad se apreció que prevalecieron por igual (34,5 por ciento) los grupos de pacientes con enfermedad en su inicio y en remisión completa. La neutropenia febril (48,3 por ciento) y la sepsis respiratoria (31,1 por ciento) fueron las manifestaciones clínicas más detectadas. Los enfermos con algún grado de neutropenia resultaron los más frecuentes (55,1 por ciento), principalmente aquellos con neutropenia severa y muy severa. La resistencia a los carbapenémicos entre los infectados fue de 58,6 por ciento. 24,1 por ciento de los casos fallecieron y la resistencia a los carbapenémicos se asoció significativamente (p<0,05) a esta mortalidad. De los procederes invasivos empleados en estos pacientes, predominó la colocación de catéter centro-venoso (58,6 por ciento). Conclusiones: La infección por A. baumannii es más frecuente en pacientes con afecciones hematológicas malignas y que recibieron tratamiento mielodepresor así como en aquellos que presentaron neutropenia severa/muy severa, manifestándose clínicamente de forma mayoritaria como una neutropenia febril; es muy importante señalar que la mortalidad por este agente biológico resultó elevada, principalmente si existe resistencia a los carbapenémicos(AU)

Introduction: Infectious complications have become the most frequent cause of mortality in patients with hematological conditions subjected to aggressive therapeutic regimens. Objective: To characterize Acinetobacter baumannii infection in patients with hematological disorders. Methods: An ambispective and descriptive study was carried out in the hematology department at Hermanos Ameijeiras Clinical Surgical Hospital from January 2010 to December 2016. Twenty nine patients, who met the established inclusion criteria, formed the sample. Results: The average age was 48.3 ± 14.8 years, males were majority (65.5 percent). The lymphomas cases were more frequent (48.3 percent). Regarding the state of the disease, it was observed that the groups of patients with disease in its initial stage and in complete remission prevailed equally (34.5 percent). Febrile neutropenia (48.3 percent) and respiratory sepsis (31.1 percent) were the most detected clinical manifestations. Patients with some degree of neutropenia were the most frequent (55.1 percent), mainly those with severe and very severe neutropenia. The resistance to carbapenems among the infected subjects was 58.6 percent. The deceased cases represented 24.1 percent and resistance to carbapenems was significantly associated (p <0.05) with this mortality. Out of the invasive procedures used in these patients, central venous catheter placement was predominant (58.6 percent). Conclusions: A. baumannii infection is more frequent in patients with malignant haematological conditions and who received myeloablative treatment as well as in those who presented severe or very severe neutropenia, mainly exhibiting as a febrile neutropenia. It is very important to point out that mortality due to this biological agent was high, mainly if there is resistance to carbapenems(AU)

Sobrecarga de hierro en enfermedades hematológicas y no hematológicas: una bomba de tiempo / Iron overload in hematological and no hematological disease: a time bomb

La sobrecarga de hierro es una complicación frecuente en un número importante de enfermedades hematológicas que cursan con anemia y requieren transfusiones sanguíneas como parte de su terapia. Entre ellas se destacan la talasemia, la drepanocitosis, los síndromes mielodisplásicos, la anemia de Blackfan-Diamond, la anemia de Fanconi y la deficiencia de piruvato quinasa, La sobrecarga de hieroo tambiún se presenta en otras enfermedades tales como la hemocromatosis hereditaria, la hepatitis viral, el síndrome metabólico y determinados trastornos neurovegetativos. El diagnóstico de sobrecarga suele hacerse mediante la determinación del hierro sérico no unido a la transferrina, la ferritina sérica y un aumento de la concentración hepática de hierro. Las consecuencias más importantes del efecto tóxico de un exceso de hierro son las disfunciones cardíacas y endocrinas, debidas al efecto oxidante del hierro sobre las membranas celulares, con el consiguiente daño celular. Tales alteraciones contribuyen al incremento de la morbilidad y la mortalidad en estos pacientes. El tratamiento consiste básicamente en el usode agentes quelantes de hierro que facilitan la excreción del exceso del metal y reducen su efecto tóxico, Entre tales agentes se cuentan la deferrioxamina (de uso intravenoso). y móa recientemente el deferiprone (ambos de uso orak)

Iron overload is a frequent complication in patients with hematological diseases which develop anemía and require blood transfusion as a therapeutic measure. Thalassemia, drepanocytosis, myelodisplastic syndromes, Blackfan-Diamond anemia, Franconi anemia and pyruvate kinase deficiency are the most common of these diseases. Iron overload is the hallmark of hereditary hemochromatosis, and also complicates diseases such as viral hepatitis, the metabolic syndrome, and certain neurovegetative disfunctions. The diagnosis of iron overload is commonly established through the evaluation of serum iron, transferrin saturation, serum ferritin and liver iron concentration. Cardiac and endocrine dysfunctions are the most important consequences of the toxic efffect of iron accumulation; these are due to the oxidixing effect of iron upon the cellular membranes, followed by cellular damage. Such alterations contribute to the increased morbility and mortality rates in these patients. The treatment of iron overload is based mainly on the use of iron chelators which facilitate the excretion of iron excess and reduce its toxic effect. Deferrioxamine (for intravenous use), and more recently deferiprone and deferasirox (both for oral administration) are the drugs of choice

Clinical characteristics and the usefulness of the QuantiFERON-TB Gold In-Tube test in hematologic patients with hepatic or splenic lesions

BACKGROUND/AIMS: Hepatic or splenic lesions in hematologic patients are not defined well because they are not easy to evaluate due to limitations of invasive procedures. Management typically depends on the clinical diagnosis with few microbiological data. METHODS: We reviewed the medical records of consecutive hematologic patients with hepatic or splenic lesions in the infectious diseases unit from April 2009 to December 2010 at the Catholic Hematopoietic Stem Cell Transplantation Center in Korea. RESULTS: Twenty-six patients were identified. Their mean age was 46.0 +/- 14.7 years, and 16 (61.5%) were male. Underlying diseases were acute myelogenous leukemia (n = 15, 57.7%) and myelodysplastic syndrome (n = 6, 23.1%). Among the nine nontuberculous infectious lesions, two bacterial, six fungal, and one combined infection were identified. The numbers of confirmed, probable, and possible tuberculosis (TB) cases were one, three, and four, respectively. Two patients had concurrent pulmonary TB. QuantiFERON-TB Gold In-Tube (QFT-GIT, Cellestis Ltd.) was positive in seven cases, among which six were diagnosed with TB. The sensitivity and specificity of QFT-GIT were 75% and 81.3%. Nine (34.6%) were defined as noninfectious causes. CONCLUSIONS: Causes of hepatic or splenic lesion in hematologic patients were diverse including TB, non-TB organisms, and noninfectious origins. TB should be considered for patients not responding to antibacterial or antifungal drugs, even in the absence of direct microbiological evidence. QFT-GIT may be useful for a differential diagnosis of hepatosplenic lesions in hematologic patients.

Urgencias en Hematología: alteraciones metabólicas y leucocitarias / Emergencies in Hematology: metabolic and leukocyte alterations

Las urgencias que pueden surgir durante la evolución de las enfermedades hematológicas son diversas, tanto por el comportamiento de la enfermedad de base como por el momento en que se presentan. Pueden ser la primera manifestación de la enfermedad o aparecer en el curso de su evolución, y sus secuelas pueden ser menores si se diagnostican y tratan adecuadamente. Se hace una revisión de algunas de las alteraciones metabólicas y leucocitarias más frecuentes que se pueden presentar en el paciente oncohematológico

Emergencies which may appear during the evolution of hemopathies are diverse due to their basic behavior as well as according to the moment the disease comes into view. They may be the first sign of the disease or could appear during the course of its evolution. Consequences might be milder if diagnosed and treated properly. A revision of some of the most frequent metabolic and leukocyte alterations that could arise in the patient with an oncohematological disease is shown

Orofacial manifestation of hematological disorders: Hemato-oncologic and immuno-deficiency disorders.

The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases with special reference to hemato-oncologic, immuno-deficiency disorders, and human immunodeficiency virus infection. A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases with emphasis on hemato-oncologic and human immunodeficiency virus (HIV) infection. Mesh phrases used in the search were: Oral diseases AND hematological disorders; orofacial diseases AND leukemias; orofacial lesions AND lymphomas; orofacial diseases AND multiple myeloma, orofacial manifestations AND HIV. The Boolean operator "AND" was used to combine and narrow the searches. The full texts of these articles were thoroughly examined. References in these articles also were manually searched non-Medline articles. Only relevant articles were selected for the review. Orofacial manifestation of malignant hematological diseases may present as primary clinical features due to infiltration of orofacial tissues, or as secondary due to the subsequent infiltration of normal bone marrow elements, or tertiary due to the side effects of the treatment. HIV-associated orofacial lesion may be a clinical indicator of HIV infection in otherwise healthy, undiagnosed individuals; an early clinical feature of HIV infection; clinical markers for the classification and staging of HIV disease or may be a predictor of HIV disease progression. Orofacial manifestations of malignant hematological diseases and HIV infection are not uncommon findings in clinical practice. These manifestations may be clinical indicators of hematologic disorders in otherwise healthy, undiagnosed individuals.

Strabismus and Poor Stereoacuity Associated with Kabuki Syndrome

Kabuki syndrome is characterized by long palpebral fissures, large ears, a depressed nasal tip, and skeletal anomalies associated with postnatal dwarfism and mental retardation. There have been few prior detailed descriptions of strabismus or stereopsis in these patients. We report a patient with Kabuki syndrome who showed small-angle strabismus and poor stereopsis. This case illustrates the need for patients with a diagnosis of Kabuki syndrome to have an ophthalmologic evaluation. Strabismus associated with Kabuki syndrome may have a small angle that can be easily overlooked.

Urolithiasis in Patients Suffering from Malignant Hematologic Diseases

PURPOSE: We performed this study in order to evaluate the incidence and characteristics of urolithiasis in patients with malignant hematologic diseases. MATERIALS AND METHODS: Nine hundred one patients who underwent medical treatment for malignant hematologic disease and 40,543 patients who visited the emergency room and without malignant hematologic diseases were included in our study. The patients with malignant hematologic diseases were divided into two groups depending on their primary treatment. Group I included patients with acute and chronic leukemia (AML, ALL, CML, CLL) for which chemotherapy and steroid therapy was necessary, and group II included patients with anaplastic anemia and myelodysplastic syndrome and who had undergone repeated transfusion for treatment. Comparisons were made between the two groups in respect to the incidence of urolithiasis and the stones' radiopacity. RESULTS: Twenty nine patients (3.2%) of the 901 malignant hematologic patients were diagnosed with urolithiasis, compared to 575 patients (1.4%) of 40,543 emergency room patients. There was a significant increase of the incidence of urolithiasis in the malignant hematologic group. Compared to the general patients, the patients with malignant hematologic diseases had a higher rate of radiolucent stones (46.6% versus 16.3%, respectively), and the difference was significant. CONCLUSION: The incidence of urolithiasis for malignant hematologic patients was significantly higher than that for the control group.

Evaluation of hematological causes in cases of nasal bleeding

To find the causes of hematological disorder in patients of epistaxis attending ENT department, Abbasi Shaheed Hospital. This cross sectional study was carried out in ear, nose and throat and hematology departments of Abbasi Shaheed Hospital from Jan 2006 to Dec 2008. Patients of epistaxis attending ENT out patient deptartment and emergency were included. A questionnaire was filled including brief clinical history, general and local examination. Based on history, patients were stratified into two groups. Group 1 included patients with single episode of epistaxis while in group 2 patients with a history of recurrent epistaxis were enrolled. For hematology workup complete blood count with peripheral film examination, bleeding time, prothrombin time and activated partial thromboplastin time were done. Specialized test like platelet aggregation test, VonWillibrand factor Ag, Ristocetin cofactor and factor VIII assay were carried out in selected cases. Bone marrow aspiration and trephine biopsy were done for the workup of cytopenia. The causes of bleeding assayed and recurrent epistaxis were compared with single episode. 10% patients were having various hematological disorders. In majority of cases no cause was found. Recurrent epistaxis or epistaxes with mucocutaneous bleeding were found to be more associated with hematological disorder and in the absence of local pathology it should be thoroughly investigated

Autopsy-proven causes of death in lungs of patients immunocompromised by secondary interstitial pneumonia

PURPOSE: To present the more frequent associations found in autopsies of immunocompromised patients who developed secondary interstitial pneumonia as well as the risk of death (odds ratio) in having specific secondary interstitial pneumonia according to the cause of immunocompromise. METHOD: From January 1994 to March 2004, 17,000 autopsies were performed at Hospital das Clínicas, São Paulo University Medical School. After examining the pathology report review, we selected 558 of these autopsies (3.28 percent) from patients aged 15 years or more with primary underlying diseases who developed radiologically diffuse infiltrates of the lung during their hospital course and died after secondary interstitial pneumonia (bronchopneumonia, lobar pneumonia, interstitial pneumonia, diffuse alveolar damage, pulmonary recurrence of underlying disease, drug-induced lung disease, cardiogenic pulmonary edema, or pulmonary embolism). Histology slides were reviewed by experienced pathologists to confirm or not the presence of secondary interstitial pneumonia. Statistical analysis included the Fisher exact test to verify any association between histopathology and the cause of immunocompromise; a logistic regression was used to predict the risk of death for specific histological findings for each of the independent variables in the model. RESULTS: Secondary interstitial pneumonia was histologically represented by diffuse interstitial pneumonitis ranging from mild nonspecific findings (n = 213) to a pattern of diffuse alveolar damage (n = 273). The principal causes of immunocompromise in patients with diffuse alveolar damage were sepsis (136 cases), neoplasia (113 cases), diabetes mellitus (37 cases), and transplantation (48 cases). A high risk of death by pulmonary edema was found for patients with carcinoma of colon. Similarly, in patients with lung cancer or cachexia, A high risk of death by bronchopneumonia (OR = 3.6; OR = 2.6, respectively) was found. Pulmonary...

OBJETIVO: Apresentar as associações mais freqüentes encontradas em autópsias de pacientes imunossuprimidos que desenvolveram pneumonia intersticial secundária bem como o risco de óbito (Odds Ratio) de desenvolver PIS associada à causa da imunossupressão. MÉTODO: De janeiro de 1994 a março de 2004, 17000 autópsias foram realizadas no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. A partir da revisão dos laudos patológicos foram selecionados 558 destas autópsias (3,28 por cento) de pacientes com 15 anos de idade ou mais, com alguma doença de base que desenvolveu um infiltrado pulmonar radiologicamente difuso durante o curso da hospitalização e que depois foi para óbito com pneumonia intersticial secundária (broncopneumonia, pneumonia lobar, pneumonia intersticial, dano alveolar difuso, doença pulmonar recorrente, doença pulmonar induzida por drogas, edema pulmonar cardiogênico e embolismo pulmonar). As lâminas histológicas foram revisadas por patologistas experientes para confirmar ou não a presença de pneumonia intersticial secundária. A análise estatística incluiu o "Teste exato de Fisher" para verificar associação entre a histolopatologia e causa de imunocomprometimento; e regressão logística para predizer o risco de óbito por achados histológicos específicos para cada variável independente do modelo. RESULTADOS: A pneumonia intersticial secundária foi representada histológicamente por pneumonite intersticial difusa variando de características não especificas leves (n=213) ao padrão histológico de dano alveolar difuso (n=273). A principal causa de imunossupressão nos pacientes com dano alveolar difuso foi sepse (136 casos), neoplasia (113 casos), diabetes melito (37 casos) e transplantados (37 casos). O maior risco de morte por edema pulmonar foi encontrado nos pacientes com carcinoma de cólon. Da mesma forma, nos pacientes com câncer pulmonar ou cachexia ocorreu um alto risco de morte (OR=3.6; OR=2.6, respectivamente)...