Hematological Characteristics Of Patients Research Articles

P1526: AUTOIMMUNE HEMOLYTIC ANEMIAS: RELATIONSHIP BETWEEN SINGLE NUCLEOTIDE POLYMORPHISMS OF CYTOKINE GENES AND CLINICAL/HEMATOLOGICAL PARAMETERS

Background: Autoimmune hemolytic anemia (AIHA) is highly heterogeneous and involves several pathogenic mechanisms including autoantibody production, complement activation, and cytokine dysregulation. Hb levels at baseline and the degree of bone marrow compensation are the main predictors of outcome. The contribution of high, intermediate, and low producer genetic profile cytokine genes may give further insights into the pathogenesis of the disease. Aims: To evaluate the frequency of cytokine single nucleotide polymorphisms (SNPs) in warm (wAIHA) and cold agglutinin disease (CAD) and their relationship with clinical and hematological parameters, including the degree of anemia, treatment refractoriness and number of therapies lines. Methods: We investigated SNPs of five cytokine genes [tumour necrosis factor (TNF)-alpha, interferon (IFN)-gamma, transforming growth factor (TGF)-beta, interleukin (IL)-6 and IL-10] in 72 Italian warm AIHA patients, using the Cytokine Tray (One Lambda, Canoga Park, CA) according to the manufacturer’s recommendations. Allele and genotype frequency results were compared to those described in the Italian population by Uboldi de Capei et al. (2003) and Poli et al. (2002). Results: One hundred and twenty AIHA patients have been evaluated: median age 63 years (range 17-90), classified as wAIHA (n=75) and CAD (n=45). Demographic and hematological characteristics of patients are described in Figure 1A. Regarding cytokine SNP frequencies (Figure 1B), we observed that the TNF-alpha-308 high producers genotypes (G/G and G/A) were significantly more frequent in AIHA patients (37% vs 16%, p=0.0003 or 37% vs 23%, p=0.01) and in wAIHA (43% vs 16%, p<0.0001 or 43% vs 23%, p=0.0014) compared to controls. No differences were observed in CAD patients versus controls. Likewise, no significant differences between AIHA (including wAIHA and CAD subtypes) and controls were noted regarding other cytokine SNPs. Concerning the clinical severity at presentation, the frequency of TGF-beta (82% vs 58%, p=0.01) and IL-10 (17% vs 5%, p=0.03) high producers was higher in patients with Hb ≤7.5 g/dL (median of all AIHA) versus those with less severe presentation. Similarly, the frequency of high/intermediate IFN-gamma producers was higher within the group with Hb levels ≤7.5 g/dL at onset (75% vs 58%, p=0.03). No characteristic patterns were observed regarding IL-6 and TNF-alpha. Finally, the patterns of TNF-alpha and TGF-beta production were differently distributed considering the number of therapy lines: the first both in all AIHA and in CAD patients (p=0.004 and p=0.01, respectively), the second only in CAD patients (p=0.03). No differences in genotype frequencies of cytokine SNPs were observed regarding the number of relapses. Image:Summary/Conclusion: These preliminary data show that AIHA patients display a more pro-inflammatory cytokine signature at molecular level (TNF-alpha high producer genotype) as compared to the Italian control population. Moreover, cytokine SNPs were also associated with clinical severity at onset, and requirement of multiple treatments.

Single Nucleotide Polymorphisms of Cytokine Genes in Warm Autoimmune Hemolytic Anemias: Relationship with Clinical and Hematological Parameters

Background: Autoimmune hemolytic anemia (AIHA) is due to an increased immune peripheral destruction of red blood cells. The disease is characterized by high heterogeneity regarding anemia severity, treatment requirement and occurrence of complications. It has been shown that Hb levels at baseline, presence of complement activation, and the degree of bone marrow compensation are the main predictors of outcome. Moreover, multi-relapsing cases show bone marrow dysmyelopoiesis, and fibrosis suggesting an immune attack against bone marrow precursors. An interesting area to investigate is the genetic landscape of AIHA, including cytokine gene signature and other molecular mechanisms of refractoriness.Aim: To evaluate the frequency of cytokine single nucleotide polymorphisms (SNPs) in warm AIHA and their correlation with clinical and hematological parameters, including the degree of anemia, treatment refractoriness and number of therapies lines.Methods: We investigated SNPs of five cytokine genes [tumour necrosis factor (TNF)-alpha, interferon (IFN)-gamma, transforming growth factor (TGF)-beta, interleukin (IL)-6 and IL-10] in 72 Italian warm AIHA patients, using the Cytokine Tray (One Lambda, Canoga Park, CA) according to the manufacturer's recommendations. Allele and genotype frequency results were compared to those described in the Italian population by Uboldi de Capei et al. (2003) and Poli et al. (2002).Results: Seventy-two warm AIHA patients have been evaluated: median age 63 years (range 17-90), classified as per DAT positivity in IgG+ (N=48), IgG+C (N=17), IgA+ (N=1), and DAT negative AIHA (N=6). Demographic and hematological characteristics of patients are described in Figure 1A. Regarding cytokine SNP frequencies, we observed that AIHA patients had a significantly higher prevalence of the TNF-alpha-308 high producers genotypes (G/G and G/A) compared to controls (40.3% vs 16% or 23%, p=0.0003 and p=0.006, respectively). No differences were detected regarding genotype frequencies of the other cytokine SNPs (Figure 1B). By dividing patients according to anemia severity at onset, a more frequent low producer genotype of IFN-gamma (A/A) was observed in patients with Hb levels >7.4 g/dL (median value of patients cohort) compared to the other group (p=0.05). Finally, considering the number of relapses or therapy lines, an increased frequency of TNF-alpha -308 high producers genotypes were found among multi-relapsing (> 3 relapses, 70.5% vs 56%) and multi-treated patients (> 3therapy lines, 52% vs 35%), although not significantly.Conclusions: These preliminary data show that wAIHA patients display a more pro-inflammatory cytokine signature at molecular level as compared to the Italian control population. This is highlighted by an increased prevalence of TNF-alpha high producer genotype that is also associated with multiple relapses and greater treatment requirement. Additionally, cytokine SNPs may be also associated with anemia severity at onset, as observed for IFN-gamma low producer genotype that was more frequent in less severe cases. [Display omitted] DisclosuresFattizzo: Alexion, Amgen, Annexon, Apellis, Grifols, Novartis, Momenta: Consultancy, Speakers Bureau. Bianchi: Agios pharmaceutics: Consultancy, Membership on an entity's Board of Directors or advisory committees. Barcellini: Agios: Honoraria, Research Funding; Bioverativ: Membership on an entity's Board of Directors or advisory committees; Novartis: Honoraria; Alexion Pharmaceuticals: Honoraria; Incyte: Membership on an entity's Board of Directors or advisory committees.

Clinical and Hematological Characteristics of Patients with COVID-19 Co-infected with Bacteria

Background: The pandemic caused by the new coronavirus is overwhelming the world. Bacteria and fungi were detected in some cases, which suggested the associations between COVID-19 and bacterial and fungal infections. Objectives: To provide suggestions and treatment opinions by analyzing laboratory data of COVID-19 patients co-infected with bacteria. Methods: We analyzed 63 patients with COVID-19 admitted to the isolation ward of the First Affiliated Hospital of Wenzhou Medical University. COVID-19 was detected using PCR, and bacteria were identified using culture. Patients were divided into two groups, including those with and those without bacterial infections, and differences in hematologic indices between the groups were analyzed. Results: There were 63 patients with median age of 55.82 years. The average hospital stay was 22.56 days. Seven patients (11.11%) had coincident bacterial infections. Detection rates in sputum/alveolar lavage and blood were the highest, 60.52% and 21.05%, respectively. Klebsiella pneumoniae, Acinetobacter, and Stenotrophomonas maltophilia were the most common found in 31.58%, 18.42%, and 15.79%, respectively. Interleukin 6 (IL-6) levels were elevated in 84.13% of patients, while IL-10 levels were elevated in 69.84%, blood ammonia levels were elevated in 82.05%, lactate levels were elevated in 75.41%, and LDH levels were elevated in 69.84%. There were significant differences between the groups in terms of expression levels of IgG, C4, AST, LDH, IL-6, IL-10, percentage of neutrophils, percentage of lymphocytes, and platelets. Conclusions: For patients with COVID-19 suspected of having bacterial infections, empiric antibiotics should be given to cover K. pneumoniae, Acinetobacter, and S. maltophilia.

Hematological characteristics of patients with novel coronavirus pneumonia in intensive care unit

BackgroundToward the end of December 2019, a novel type of coronavirus (2019-nCoV) broke out in Wuhan, China. Here, the hematological characteristics of patients with severe and critical 2019-nCoV pneumonia in intensive care unit (ICU) were investigated, which may provide the necessary basis for its diagnosis and treatment. MethodsWe collected data on patients with confirmed 2019-nCoV pneumonia in the ICU of Leishenshan Hospital in Wuhan from February 25 to April 2, 2020. Real-time reverse-transcription polymerase chain reaction was used to confirm the presence of 2019-nCoV, and various hematological characteristics were analyzed. ResultsAll patients tested positive for 2019-nCoV using nasopharyngeal swabs or sputum after admission, and interstitial pneumonia findings were noted on chest computed tomography. Sex, age and comorbidities were not significantly different between the severe and critical groups. In terms of prognosis, the survival rate of patients in the severe group reached 100%, whereas that of patients in the critical group was only 13.33% after positive treatment. Furthermore, lymphocyte percentage, blood urea nitrogen, calcium, D-dimer, myohemoglobin, procalcitonin, and IL-6 levels were high-risk factors for disease progression in critical patients. Finally, lymphocyte percentage and blood urea nitrogen, calcium, myohemoglobin, and IL-6 levels were closely associated with patient prognosis. Conclusions2019-nCoV pneumonia should be considered a systemic disease. Patients with more complications were more likely to develop critical disease. Lymphocyte percentage and blood urea nitrogen, calcium, myohemoglobin, and IL-6 levels can be monitored to prevent progression critical disease.

Hematological characteristics of patients in coronavirus 19 infection: a systematic review and meta-analysis

ABSTRACT Background COVID-19 infection has become a pandemic and a global health issue since its origin in Wuhan, China in December 2019. The present systematic review and meta-analysis aim to assess hematological changes seen in COVID-19 infection and their association with the severity of the disease. Methods Pooled proportions were calculated using both fixed effects model and random effects model. Weighted mean difference and 95% CI were calculated and reported. Results Initial search identified 84 reference articles, 23 relevant articles were selected and reviewed. Compared to general population, the weighted mean difference of WBC count in all COVID-19 patients was lower by 0.97 × 109 mm3 (95% CI = –1.29 to –0.66). In severe COVID-19 patients, the weighted mean difference of platelet count was lower by 23.85 × 109/liter (95% CI = –35.18 to –9.53), as compared to general population. The weighted mean difference of prothrombin time, D-Dimer, and fibrinogen in severe COVID-19 patients was higher by 1.92 seconds (95% CI = 0.01 to 3.84), 6.23 mg/liter (95% CI = 0.11 to 12.36) and 1.88 g/liter (95% CI = 1.18 to 2.48) respectively, as compared to general population. Pooled proportion showed D-Dimer to be elevated in 80.00% (95 % CI = 50.00 to 99.00) of severe patients. Conclusions Our meta-analysis shows that patients with COVID-19 have significant thrombocytopenia, leukopenia along with elevated D-dimer, fibrinogen and prothrombin time. These laboratory findings are marked in severe COVID–19 infections and could be helpful in early recognition of severe infection.

Viral Hepatitis in Dhi-Qar Province: Demographics and Hematological Characteristics of Patients

Viral Hepatitis in Dhi-Qar Province: Demographics and Hematological Characteristics of Patients

Hematologic Characteristics of Patients with Active Pulmonary, Extra-Pulmonary and Disseminated Tuberculosis: A Study of over Six Hundred Patients

Background: Many inflammatory cells, cytokines, acute phase reactants as well as platelets are recruited in the battle against the invading mycobacterium. As a result, alterations in the hematologic profile of infected patients are anticipated. Objectives: The primary objective was to investigate the various hematologic characteristics of patients with active tuberculosis. The secondary objective was to study the correlation between such hematologic characteristics and the type of tuberculosis including pulmonary, extra-pulmonary, and disseminated. Methods: This was a retrospective, descriptive study investigating the hematological findings in adult patients (aged 18 years or older) with active, bacteriologically-confirmed tuberculosis infection. Results: Among the 605 confirmed active tuberculosis cases, 465 (78.8%) were pulmonary, 104 (17.6%) extra-pulmonary, and 21 (3.6%) disseminated type. The mean age at diagnosis was 33.4 ± 11.4 years and males constituted 80.2% of cases. Peripheral leukocytosis was observed in 177 (30.1%) and leukopenia in 7 (1.2%) (Pulmonary type of tuberculosis was significantly associated with leukocytosis (P = 0.000)). Neutrophilia, lymphocytosis, monocytosis, eosinophilia, and basophilia were observed in 15.3%, 3.1%, 10.4%, 6.3% and 4.7% respectively. Thrombocytopenia and thrombocytosis were observed in 3.9% and 26.7% respectively. Anemia was observed in 44.8% of all cases with 4.4% had severe anemia and 60.3% had anemia of chronic diseases. Erythrocyte sedimentation rate was high in 77.2% of cases and ≥100 mm/hr in 5%. C-reactive protein was high in 93.4% and it exceeded 40 mg/L in 69.3%. Conclusion: There is a wide range of variability in the leukocyte and differential leukocyte abnormalities observed in patients with active tuberculosis. Leukocytosis can be seen in one-third of patients with pulmonary tuberculosis. Anemia of chronic diseases is the most common type of anemia observed in tuberculosis patients. Erythrocyte sedimentation rate and C-reactive protein are elevated in the majority of patients with active tuberculosis. Levels of the sedimentation rate can be useful indices to determine the extent of the disease.

Bone Marrow Cytomorphology And Clinico-Hematological Profile of Metastatic Solid Tumors: A 5-Year Audit from A Tertiary Care Centre in Northern India

Introduction: Metastasis to bone marrow by solid malignancies is a rare event and it upgrades the tumor thereby worsening the prognosis. This is caused by an impaired response to therapy and other manifestation resulting from decreased hematopoiesis. The common malignancies to metastasize in male and female are carcinoma prostate and carcinoma breast, respectively. It is noted that metastasis from small round cell tumor (SRCT) predominates in children. Here, we present a series of cases of bone marrow metastasis by solid organ malignancies over a period of 5 years. Objectives: The aim of this study was to investigate the clinical and hematological characteristics of patients with BM metastases of solid tumors diagnosed by bone marrow (BM) aspiration. Materials & methods: In this study, we reviewed 3520 bone marrow aspirates in our hospital from January 2013 to December 2017. Patients diagnosed with solid malignancies metastasizing to bone marrow were selected. Results: We found a total of 26 cases of solid malignancies with metastasis to bone marrow. The age of the patients ranged from 2–88 years (mean age of 46 years). Most of the patients were adults (19/26), of which 77.7% were males. Seven of the 26 patients were in the pediatric age group, with an age range of 2-11 years. Various malignancies metastasizing to marrow were evaluated and clinical and hematological correlation was done. The common presenting sign & symptoms were fever, anorexia and bodyache and organomegaly. Anemia was the most common hematological finding at the time of admission. Carcinoma prostate was the most common metastatic tumor in adults and small round cell tumor in children. Conclusion: BM is one of the important and infrequent sites of metastasis of solid tumors. Detection of bone marrow metastasis has significant clinical implication with impact on therapeutic decisions and is associated with poor prognosis. Aspiration of the BM provides an easy and quick way of detecting involvement, however, biopsy with immunohistochemical and/or molecular techniques may be needed in case of microscopical tumor burden.

Clinical and Hematological Characteristics of Patients with Chronic Myeloid Leukemia under Present-Day Conditions: Results of the Russian Part of International Multi-Center Prospective EUTOS Population-Based CML Study

Background. Much attention has been paid to molecule-genetic diagnostics of chronic myeloid leukemia (CML) and its treatment using new highly effective methods of therapy. The baseline characteristics of patients at primary CML diagnosis are hardly discussed in literature. Aim. To provide clinical, hematological, molecular genetic and demographic characteristics of patients obtained at primary diagnosis of CML. Patients &amp; Methods. Characteristics of CML patients are based on data gathered by the Russian Investigational Group for CML within the international project European Treatment and Outcome Study of CML in Europe (EUTOS, the European Treatment and Outcomes Study). The study included 197 patients with newly diagnosed CML in 6 regions of the Russian Federation (Mordovia, Kirov, Perm (2 sites), Bryansk, Irkutsk, and Chita) over the period from 2009 till 2012. Results. The study demonstrated that 94 % of CML cases were diagnosed in the chronic phase (CP) and 6 % of cases in the acceleration phase (AP) and the blast crisis phase (BC). In 40 % of patients there were no clinical symptoms, and CML was suspected only due to changes in the CBC test. Fatigue was the main subjective complaint presented by 77 % of patients in the CP and 100 % of patients with the AP and BC. Peripheral blood leukocytosis, left shift to immature myeloid cells and increased granulocytic lineage in bone marrow were typical for the patients. In all patients, the CML diagnosis was confirmed by cytogenetic or molecular tests. The social and demographic characteristics of CML patients and comorbidities at diagnosis were analyzed. Conclusion. Based on the results of the study, a modern «portrait of a CML patient» was obtained. The study demonstrated that cytogenetic and molecular methods allow to diagnose CML in most patients at early stages of the disease in the absence of clinical signs of progression. The data on comorbidities require a special attention while choosing a therapy considering its duration. Demographic and social characteristics of CML patients demonstrate that they are socially active, particularly interested in retaining the working capacity and quality of life.

Clinical and hematological characteristics of patients with multiple

The article presents data on the frequency and characteristics of clinical manifestation of non-Hodgkin’s lymphoma, and the results of the study characteristics of peripheral blood in patients with nonHodgkin’s lymphoma. We discuss the pathogenic mechanisms of symptoms and syndromes in patients with non-Hodgkin’s lymphoma.

THE RESULTS OF TREATMENT OF ACUTE MYELOID LEUKEMIA IN EVERYDAY CLINICAL PRACTICE

Aim: To assess efficacy of the “7 + 3” regimen in non-selective groups of patients with acute myeloid leukemia (AML). Materials and methods: We retrospectively assessed medical documentations (inand out-patient case histories) of 105 AML patients who were on treatment in MONIKI from January 2002 to December 2011, with evaluation of clinical and hematological characteristics of patients, remission rates depending on age, serum lactate dehydrogenase (LDH) levels and time from diagnosis to the start of treatment, as well as overall survival. Results: In total, the standard regimen “7 + 3” was effective in 58% of AML patients. Remission was achieved in 70.5% of 17 patients with normal LDH levels and in 50.9% of 53 patients with high LDH levels (р < 0,05). In the patients aged from 18 to 30 years, the remission rate was 81%, in those from 31 to 60 years, in 61%. In the elderly patients (from 60 years and older), remission was achieved in 29% of cases (р < 0.05 compared to each of two other age groups). Remission was associated with the time from diagnosis to the start of treatment, being 16.7% in those where this time period exceeded 10 days and 60.8% in those where this time was less than 10 days (р < 0.05). The median overall survival was 11 months in the total group, 15 months in the patients who achieved remission after 2 treatment cycles and 6 months in those who did not respond to treatment. Comparison of the 2 groups with different AML types did not reveal any difference in the treatment results: overall survival in М0–М2 was 11 months and in М4–М5 10 months. Conclusion: Treatment of AML patients with “7 + 3” regimen provides a good anti-tumor effect. Achievement of remission depends on patient age, time to the start of treatment and LDH levels. The results obtained in non-selective group of patients are comparable to those published on selected group of patients.

The body composition of patients with knee osteoarthritis: relationship with clinical parameters and radiographic severity.

We investigated body composition in knee osteoarthritis (OA) patients and evaluated its relationship with clinical parameters and radiographic severity. Sixty-four patients with knee OA (52 females and 12 males with a mean age of 57.7±8.6years) and thirty healthy volunteers (20 females and 10 males with a mean age of 56.3±9.5years) were evaluated. Controls were selected among similar to demographic and hematologic characteristics of patients. Body compositions were assessed via bioelectrical impedance analysis (BIA). Each patient was clinically evaluated by the Western Ontario and McMaster University Osteoarthritis Index (WOMAC). In addition, radiographic severity was classified according to Kellgren-Lawrence's criteria. Phase angle, body capacitance, resistance, reactance, lean body mass, and intracellular water values of the patients with knee OA were found to be significantly lower than those of the controls (p<0.05). Furthermore, fat mass and extracellular water levels were significantly higher in the patients compared to the controls (p<0.05). Lean body mass was inversely correlated with WOMAC score (r=-0.716, p<0.001), whereas fat mass was moderately correlated with WOMAC score (r=0.281, p<0.05) in bivariate analysis. However, with respect to the body composition, there was no significant difference between early grades and late grades in the knee OA with patients (p>0.05). Body composition assessed using BIA might be associated with knee OA, and be a noninvasive tool for diagnosis of knee OA. However, body composition may not be predictive of the progression of knee OA.

Demographic, clinical, and hematologic characteristics of patients with chronic myeloid leukemia in Upper Egypt: association with treatment responses

Background and objectives Chronic myeloid leukemia (CML) is a relatively indolent hematologic malignancy that carries poor prognosis if left untreated. With the recent advances in treatment options for CML, therapy could be tailored to each patient based on patient and/or disease characteristics. This research aimed to study the characteristics of patients with CML in Upper Egypt and to investigate their influence on various therapeutic responses. Patients and methods A retrospective study was conducted at the Hematology Unit of Assiut University Hospital and South Egypt Cancer Institute. The demographic, clinical, hematologic, and follow-up data of patients with CML were extracted from hospital records at both Assiut University Hospital and South Egypt Cancer Institute during the period from January 2007 to December 2012, representing a total of 180 patients. Records with incomplete data or unavailable follow-up were excluded from the study.Results and conclusion The median age of participants was 42 years, and the male-to-female ratio was 1 : 1.7. The Eastern Cooperative Oncology Group Performance Status was the most important effector of both hematologic and therapeutic responses (P = 0.000), followed by the phase of the disease (P = 0.000 and 0.017, respectively), and lastly the Philadelphia chromosome (P = 0.07 and 0.000, respectively). Moreover, leukocytosis was associated with poor hematologic and cytogenetic responses (r = 0.19, P = 0.000; r = −0.16, P = 0.05). A small proportion of patients achieved complete hematologic and cytogenetic responses (45 and 52%, respectively). These novel findings may reflect a trend of young age, female-predominant CML in Upper Egypt and could be attributed to the ethnic and socioeconomic differences of our patients compared with those in similar studies.

뇌졸중 환자의 체질별 혈액학적 특성 -1개 한방병원의 입원환자를 중심으로-

Objectives The purpose of this study is to know hematologic characteristics of patients with stroke in Sasang Constitutional Medicine which will be helpful for the prevention and treatment of stroke. Methods We collected data from 111 patients who received treatment as a stroke inpatient at one Korean medicine hospital in Busan, Korea. A Sasang Constitutional Medicine(SCM) specialist diagnosed their Sasang Constitutional types based on the four diagnosis standard of SCM and herbal medicine reaction. Results of hematologic tests were classified according to gender, age, stroke types and Sasang Constitutional types and analysed by SAS system. Results 1. Male's mean value of RBC count, Hemoglobin and Hematocrit was lower than normal range. 2. The mean value of hsCRP of above age 65 group was 3.19 times significantly higher than under age 65 group. 3. The mean value of platelet of cerebral hemorrhage group was significantly higher than cerebral infarction group. 4. The mean value of triglyceride of Taeeumin was significantly higher than Soyangin and Soeumin. Conclusions For the prevention of stroke, appropriate treatment of stress, trauma and infections is needed and male require a regular and balanced meals. Especially Taeeumin need to eat small meals to avoid excessive accumulation of energy.

Preoperative diagnosis of pelvic actinomycosis by clinical cytology

BackgroundThe purpose of this work was to investigate whether clinical cytology could be useful in the preoperative diagnosis of pelvic actinomycosis.MethodsThis study involved the prospective collection of samples derived from the endometrium and the uterine cervix, and retrospective data analysis. Nine patients with clinically diagnosed pelvic actinomycosis were enrolled. The clinical and hematological characteristics of patients were recorded, and detection of actinomyces was performed by cytology, pathology, and bacteriological culture of samples and by imprint intrauterine contraceptive device (IUD) cytology.ResultsThe detection rate of actinomyces was 77.7% by combined cervical and endometrial cytology, 50.0% by pathology, and 11.1% by bacterial culture.ConclusionThe higher detection rate of actinomyces by cytology than by pathology or bacteriology suggests that careful cytological examination may be clinically useful in the preoperative diagnosis of pelvic actinomycosis.

Hematological Characteristics of Patients with Myelodysplastic Syndromes in the Light of WHO 2008 Proposals.

Myelodysplastic syndromes have been reclassified by new WHO proposals published in 2008. So far, there are no data on haematological features of the newly composed subtypes. We analyzed 2063 patients with MDS diagnosed according to the WHO 2008 classification. Patients were characterized by their initial haematological features. We especially payed attention towards isolated cytopeniae with regard to the new classification of MDS subcategories. Analyses were performed by the use of the MDS registry Duesseldorf and included hemoglobin level, platelets, leukocytes and karyotype (available in 1091 patients) as well as age, gender, IPSS and WPSS at the time of diagnosis. All items have been assessed in our haematological laboratory and in the department of human genetics, respectively. The table shows characteristic features of MDS patients according to their WHO subtype. The patients' median age did not differ substantially. There was a preponderance of females in the del(5q), RARS and RC group. Median hemoglobin level ranked lowest in the del(5q) and RAEB II group. Median thrombocyte, leukocyte as well as granulocyte level progressively decreased with more advanced WHO risk groups. Chromosomal aberrations were more frequent with increasing medullary blasts and degree of dysplasia. Likewise, patients with these characteristics were categorized into more advanced WPSS subtypes. About 90% of all patients were anemic, that is patients with a hemoglobin level of under 12 g/dl. Leukocytopenia as well as thrombocytopenia were more severe with increasing medullary blast counts. Of interest, 46% of RC patients showed a leukocytopenia (<4000/μl) and 37% had thrombocytopenia (<150.000/μl). 26% of this group showed an isolated anemia, whereas only 1–3% of the patients presented with an isolated leukocytopenia or thrombocytopenia. The majority of MDS patients presents with bicytopenia, regardless of subtypes. The degree of hematopoietic insufficiency is strongly correlated with the amount of medullary blasts, but as well with the degree of dysplasia in blood and marrow. An inclusion of the number of cytopenia into the WHO classification would lead to an alteration of subcategories and result in a shift towards a higher percentage of multilineage MDS.RCRARSMDS with del(5q)RCMDRAEB IRAEB IIFrequency18416268950306393Age707064726967Gender (%)M484835555856W525265454244Hemoglobin(g/dl)9,59,599,29,29Thrombocytes(1000/μl)1242932841469680Leukocytes(/μl)480055004300390031002800Granulocytes195032002400220014001200ChromosomalLow708796635751risk by IPSSIntermediate19102181619(%)High1132192730Initial IPSS (%)048837234001421426532721032135550300004043Initial WPSS04873403000(%)130276040002220030330300005059400001841Cytopenia (%)Anemia (<12 g/dl)859798898690Leukopenia461839506489(<4000/μl)Thrombocytopenia3738355280(<150.000/μl)Isolated (%)Anemia26785127147Leukocytopenia1,501121Thrombocytopenia300232Pancytopenia2236243544(%)Survival (mths.)5616980361910AML after33151532633 yrs. (%)

Sickle-Cell Disease in Brazzaville, Congo: Genetical, Hematological, Biochemical and Clinical Aspects

Clinical, hematological and molecular features of 116 unrelated sickle-cell anemia patients from Brazzaville were investigated. The mean age of the patients was 9.4 ± 5.3 years. 232 β^s-chromosomes were haplotyped and almost all sickle cell anemia patients (91%) were homozygous for the Bantu haplotype. All hematological indices were similar for males and females. Mean hemoglobin (Hb) concentration was 6.6 ± 1.4 g/dl. Fetal Hb (HbF) levels were from 1 to 28%, with a mean of 8.8 ± 5.8%. There was a curvilinear relationship between %HbF and age suggesting that HbF level had a selective effect on the survival of patients. This effect was sex dependent. The (–α^3.7) gene frequency was 0.45 and was not affected by stratification for age. Hematological characteristics of patients with (–α/αα, –α/–α) and without (αα/αα) the –α^3.7 α-thal-2 deletion showed trends similar to those reported in Jamaican and US sickle cell anemia patients. Hyperbilirubinemia (>38 μmol/l) was common and high lactic dehydrogenase (LDH) concentrations were recorded. Bilirubin concentrations for males and females were similar whereas those for LDH concentration were not. Hepatomegaly and splenomegaly were common. Splenomegaly was strongly associated with the –α^3.7 α-thal-2 deletion. These clinical and hematological observations indicate a more severe form of hemolytic disease in Congolese SS patients.