The recent WHO classification for acute myeloid leukemias (AML) separates entities by recurrent cytogenetic abnormalities and immunophenotypic features presenting prognostic impact. We have examined the expression of several lineage and maturation linked antigens used in routine immunophenotyping of patients with de novo AML, using a 3-color two-step panel. Cases were diagnosed by peripheral blood counts, bone marrow cytology, cytochemistry, cytogenetics and immunophenotyping (CD2, CD3, CD7, CD19, CD13, CD33, myeloperoxydase -- MPO, CD14, CD15, HLA-DR, CD34, CD56 and CD45). Antigen expression was measured by mean fluorescence intensity (MFI) by flow cytometry (Paint-a-gate software). Thirty five patients were analyzed. Median age: 51 years (15-79). Predominant FAB types were M2 and M4. In 6 cases more than one phenotypically distinct blast subpopulation could be detected. Although our set was small, we tried to analyze the impact of MFI of the examined antigens on the overall survival of the patients. In Cox univariate analysis, age, peripheral leukocytes (WBC) at diagnosis, MFI of CD45, and MPO were significant for worse a survival. In the multivariate analysis only MFI of CD45 and WBC remained in the model (p=0.018 and p=0.014 respectively). After bootstrap resampling, MFI of CD45 entered the model in 69%, WBCin 60%, age in 42% and MFI of MPO in 35% of the sets. Analysis of antigen expression by MFI permitted to detect cases presenting phenotypically distinct blast subpopulations. This may represent a pitfall in studies of minimal residual disease by flow cytometry, as chemotherapy may select one of these subsets.

We describe a case of oral metastasis of peripheral primitive neuroectodermal tumor (pPNET) in a 68-year-old man, who presented the primary lesion in the chest. Oral metastasis of pPNETs is very rare and we have not found any similar case reported in the English literature. Clinical examination showed an extensive and ulcerated fleshy mass measuring 3.0 x 3.5 cm in the right lower gingivae. Microscopic examination showed sheets of proliferating small, hyperchromatic, round cells. Tumor cells were reactive to neuron-specific enolase (NSE), vimentin and MIC-2 gene by immunohistochemistry, consistent with PNET. The patient died 3 weeks later because of respiratory insufficiency.

The Ankyrin (ANK)-repeat is one of the most common protein sequence motifs, which leads itself to variation in overall domain size by simple sequence duplication or deletion. The Mask (Multiple Ankyrin Repeats Single KH domain) gene, which codifies an ANK-repeat protein, is located in chromosome 5(q31.3) and it is composed of 39 exons. It generates isoforms by alternative 3′splicing. The first splice variant (hMask) lacks the 10A exon of the Mask gene, generating a mRNA containing 34 exons. The other, Mask-BP3ARF, results from fusion of splice variant hMask, with the two last exons of the gene Eif4Ebp3 (exons B and C) and an intermediate exon (exon 0), generating 36 exons. Recently, a new splice variant, denominated PP2500, was deposited in the data base GeneBank, it presents the first 10 exons, homologous to Mask mRNA with a poly(A+) signal and it is a new splice variant of Mask. In Drosophila, MASK protein seems to interact with members of the Receptor Tyrosine Kinase (RTK) signalling pathway and loss of this interaction increases programmed cell death, reduces cell proliferation, inhibits photoreceptor differentiation, affects RTK dependents processes but does not affect MAPK (Mitogen Activated Protein Kinase) activation. However, the biological functions of these proteins in humans remain still unknown. The aim of this study was to investigate the expression of Mask splice variants in multiple myeloma (MM). Fifteen patients with MM and 3 normal donors participated in this study. Total RNA was extracted from positively selected plasma cells in magnetic column, by Macs Microbeads antibody anti-CD138 and the percentage of purity of plasma cells varied from 78.38% to 96.02% (average 87.95%). We used as control, total RNA from positively selected plasma cells, from a culture of B normal lymphocytes of bone marrow donors (purity 88.69%). The complementary DNA (cDNA) was analyzed by Real-time detection of amplification, performed in an ABI 5700 Sequence Detector System using SybrGreen PCR Master Mix (qPCR). The b-actin gene was used as endogenous control of the reaction. The mean expression of the mRNA of the hMask and Mask-BP3ARF genes were 3 and 4 times increased, respectively, compared with control. The mean expression of the PP2500 mRNA was 14 times increased, compared with control. Quantification of hMask, Mask-BP ARF and PP2500 mRNA was not influenced by age, gender, ethnic origin, stage of the disease, β2-microglobulin, serum creatinine and lactate dehydrogenase values (Fisher's exact test, P> 0.05). Previously we demonstrated that MASK is associated with SHP2, a protein tyrosine-phosphatase. In MM, SHP2 mediates the anti-apoptotic effect of Interleukin-6 (Chauhan et al. JBC 275: 27845, 2000). Interleukin-6 triggers proliferation of MM cells via the MAPK cascade, which includes SHP2 activation. Thus, the increased expression of Mask splice variants in plasma cells of MM suggests that their proteins may be involved in this signaling pathway and provide an insight for novel treatment approaches in MM. Supported by FAPESP and CNPq

Point mutations affecting codons 12, 13 (exon 1) and 61 (exon 2) of the N-RAS gene and codons 12 and 13 (exon 1) of the K-RAS gene are identified in approximately 30.0% and 10.0%, respectively, of multiple myeloma (MM) patients living in the northern hemisphere. To date, there are no reports about the prevalence of RAS gene mutations in MM Brazilian patients, and this comprised the aim of the present study. DNA from bone marrow aspirates of 252 patients with MM (139 males and 113 females; aged 59.33 +/- 11.95 years) were investigated for whole exons 1 and 2 of the N-RAS gene and whole exon 1 of the K-RAS gene by direct sequencing of DNA amplified in vitro by the polymerase chain reaction. Fifty-three out of 252 (21.03%) MM patients presented RAS mutations. Heterozygous mutations at codons 4, 10 (exon 1), 61 and 65 (exon 2) of the N-RAS gene were identified in seven out of 252 (2.78%) patients. K-RAS heterozygous mutations at codons 7, 12, 13 (exon 1) were seen in 46 out of 252 (18.25%) patients. To the best of our knowledge, the mutation at codon 7 of K-RAS gene is reported for the first time in MM. Taken together, these results suggest that Brazilian MM patients are characterized by: (i) a low prevalence of RAS mutation and (ii) RAS mutations located at distinct regions of the critical codons of the N-RAS and K-RAS genes.

European Journal of HaematologyVolume 75, Issue 6 p. 530-531 Polymorphisms of glutathione S-transferase mu1 (GSTM1) and theta 1 (GSTT1) genes in chronic myeloid leukaemia Gustavo J. Lourenço, Gustavo J. Lourenço Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorManoela M. Ortega, Manoela M. Ortega Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorHelvia Nascimento, Helvia Nascimento Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorMaria T. Teori, Maria T. Teori Haematology and Haemotherapy Centre, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorCarmino A. De Souza, Carmino A. De Souza Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorFernando F. Costa, Fernando F. Costa Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorCarmen S. P. Lima, Carmen S. P. Lima Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this author Gustavo J. Lourenço, Gustavo J. Lourenço Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorManoela M. Ortega, Manoela M. Ortega Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorHelvia Nascimento, Helvia Nascimento Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorMaria T. Teori, Maria T. Teori Haematology and Haemotherapy Centre, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorCarmino A. De Souza, Carmino A. De Souza Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorFernando F. Costa, Fernando F. Costa Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this authorCarmen S. P. Lima, Carmen S. P. Lima Department of Internal Medicine, State University of Campinas, Campinas, São Paulo, BrazilSearch for more papers by this author First published: 10 November 2005 https://doi.org/10.1111/j.1600-0609.2005.00567.xCitations: 13 Carmen Silvia Passos Lima MD, PhD, Department of Internal Medicine, State University of Campinas, Rua Alexander Fleming no 181, Cidade Universitária ‘Zeferino Vaz’, Distrito de Barão Geraldo, Campinas, São Paulo 13083 970, Brazil Tel: +55-19-3788-7496 Fax: +55-19-3788-7496 e-mail: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. References 1 Bjork J, Albin M, Welinder H, et al. Are occupational, hobby, or lifestyle exposures associated with Philadelphia chromosome positive chronic myeloid leukaemia? Occup Environ Med 2001; 58: 722–727. 2 Hengstler JG, Arand M, Herrero ME, Oesch F. Polymorphisms of N-acetyltransferases, glutathione S-transferase, microsomal epoxide hydrolase and sulfotransferases: influence on cancer susceptibility. Recent Results Cancer Res 1998; 154: 47–85. 3 Mondal BC, Paria N, Majundar S, Chandra S, Mukhopadhyay A, Chaudhuri U, Dasgupta UB. Glutathione S-transferase M1 and T1 null genotype frequency in chronic myeloid leukaemia. Eur J Cancer Prev 2005; 14: 281–284. 4 Landi S. Mammalian class theta GST and differential susceptibility to carcinogenesis: a review. Mutat Res 2000; 463: 247–283. 5 Ruiz MA, Augusto LGS, Vassallo J, Vigorito AC, Lorand-Metze I, Souza CA. Bone marrow morphology in patients with neutropenia due to chronic exposure to organic solvents. Pathol Res Pract 1994; 190: 151–154. 6 Queiroz MLS, Bincoleto C, Perlingeiro RCR, Souza CA, Toledo H. Defective neutrophil function in workers occupationally exposed to hexachlorobenzene. Hum Exp Toxicol 1997; 16: 322–326. 7 Comstock KE, Sanderson BJ, Clafin G, Henner WD. GST1 gene deletion determined by polymerase chain reaction. Nucleic Acids Res 1990; 18: 3670–3676. 8 Pemble S, Schroeder KR, Spencer SR, Meyer DJ, Hallier E, Bolt HM, Ketterer B, Taylor JB. Human glutathione S-transferase theta (GSTT1): cDNA cloning and the characterization of a genetic polymorphism. Biochem J 1994; 300: 271–276. Citing Literature Volume75, Issue6December 2005Pages 530-531 ReferencesRelatedInformation

Fanconi anaemia (FA) is a recessive autosomal disease determined by mutations in genes of at least eleven complementation groups, with distinct distributions in different populations. As far as we know, there are no reports regarding the molecular characterisation of the disease in unselected FA patients in Brazil. OBECTIVE: This study aimed to investigate the most prevalent mutations of FANCA and FANCC genes in Brazilian patients with FA. METHODS: Genomic DNA obtained from 22 racially and ethnically diverse unrelated FA patients (mean age ± SD: 14.0 ± 7.8 years; 10 male, 12 female; 14 white, 8 black) was analysed by polymerase chain reaction and restriction site assays for identification of FANCA (delta3788-3790) and FANCC (delta322G, IVS4+4A -> T, W22X, L496R, R548X, Q13X, R185X, and L554P) gene mutations. RESULTS: Mutations in FANCA and FANCC genes were identified in 6 (27.3%) and 14 (63.6%) out of 22 patients, respectively. The disease could not be attributed to the tested mutations in the two remaining patients enrolled in the study (9.1%). The registry of the two most prevalent gene abnormalities (delta3788-3790 and IVS4 + 4 -> T) revealed that they were present in 18.2% and 15.9% of the FA alleles, respectively. Additional FANCC gene mutations were found in the study, with the following prevalence: delta322G (11.4%), W22X (9.1%), Q13X (2.3%), L554P (2.3%), and R548X (2.3%) of total FA alleles. CONCLUSION: These results suggest that mutations of FANCA and FANCC genes are the most prevalent mutations among FA patients in Brazil.

The simultaneous occurrence of two specific acquired chromosomal abnormalities in chronic or acute leukaemias is rare. Inherited chromosomal abnormalities are also rare events in the general population. In chronic myeloid leukaemia (CML), characterised by the t(9;22)(q34;q11), the inv(16)(p13q22) has been described associated with the acceleration of disease or onset of blast crisis. We report on a patient with chronic phase of CML and both acquired t(9;22)(q34;q11) and inherited inv(16)(p13q22), who obtained a complete remission of the disease after bone marrow transplant. Therefore, it is worth to comment that an additional chromosomal abnormality in disease does not obligatory mean transformation of the disease to a more aggressive form, since chromosomal abnormalities are also seen in normal individuals.

Transcription factors, including GATA-1 protein, play a key role in controlling the cellular proliferation and differentiation of hematopoietic stem cells. Inherited missense mutations in exon 4 of GATA-1 have been found in some families, leading to a variable degree of macrothrombocytopenia with or without abnormalities in the erythrocyte lineage. Acquired mutations in exon 2 of GATA-1 have been found in Down syndrome patients with transient myeloproliferative disorder (TMD) or megakaryoblastic leukemia (AMKL). These mutations prevent the synthesis of the full length GATA-1, but allow the synthesis of a smaller form of the protein (GATA-1s). Here, we report a novel inherited mutation, in exon 2 of the GATA-1 gene, which allows only the synthesis of GATA-1s in members of a family, who present anemia and neutropenia. A man, 19 years, was referred to our hospital due to anemia manifested at 4 years of age. Blood count showed: RBC: 3.0 x106/μl, HGB: 2.9g/dl, HCT: 8.3%, MCV: 94.4fl, MCH: 32.7pg, Retic: 0.6%, WBC: 2.6x103/μl, neutrophil: 1.0x103/μl, and PLT: 250.0x103/μl. The peripheral blood film demonstrated anisocytosis, macrocytosis, poikilocytosis, and neutrophils with pseudo Pelger-Hüet anomaly. The bone marrow aspirate and biopsy showed moderate hypocellularity with trilineage dysplasia. The presumptive diagnosis of hypocellular MDS was established. Karyoptype: 46, XY. DEB: negative. CFU-GM: 54 colonies/ml (normal range: 0.5 x104 to 1.0x104). Platelet aggregation test: 0.82 mg/ml (normal range: 0.7 to 1.2), platelet aggregation to 3μM adrenaline: absent (normal: present), and platelet aggregation to 3μM ADP: second wave absent (normal: present). The number of platelets expressing GPIX glycoprotein as well as those expressing GPIb, GPIIb, and GPIIIa were similar in patient and controls. No significant differences in the mean fluorescence indexes (MFI) for GPIX (1,214.0 vs 1,015.5), GPIb (154.0 vs 136.5), and GPIIb (100.0 vs 96.1) platelet contents were found in patient and controls. In contrast, the MFI for measurement of GPIIIa was slightly lower in patient than in controls (1,538.6 vs 1,928.1). Hypocellular MDS and similar laboratory findings were also identified in 6 additional males of the same family. Hemizygous and heterozygous 332G/C mutations in exon 2 of GATA-1 were found by sequencing PCR products in 7 males and in 4 phenotypically normal females of the family, comprising three generations. Only GATA-1s, probably resulting from alternative splicing of exon 2 was identified by RT- PCR and sequencing in affected males. In heterozygous females and normal members, both isoforms of the protein were found. These results indicate that the inherited 332G/C mutation in GATA-1 is associated with a clinical and hematological picture similar to that of MDS syndrome in contrast with the other mutations in exon 2 observed in Down syndrome, which are associated with AMKL or TMD.

Background: The myelodysplastic syndromes (MDS) are a group of stem cell disorders characterized by a reduction in one or more elements of the peripheral blood. Oral manifestations of the disease and oral complications of medical management may result in significant symptoms and have an impact on the systemic condition of the patient. The removal of the infectious focus, such active teeth infection or severe periodontal disease, remains controversial in these patients, due to the increased risk of bleeding and systemic infection.Methods: This paper reports a case of MDS with spontaneous gingival hemorrhage and generalized gingival hyperplasia associated with periodontal disease. This patient underwent several platelet transfusions due to these oral complications. The patient received periodontal therapy, resulting in an improvement of the oral clinical situation and a decrease of gingival hyperplasia.Results: The patient did not present any episode of gingival hemorrhage after the periodontal treatment.Conclusion: The results of this study suggest that periodontal therapy should be performed in MDS patients presenting thrombocytopenia, gingival hyperplasia, and gingival bleeding, with the intent of preventing further hemorrhagic episodes and possible systemic infection.

The role of apoptosis in the pathobiology of myelodysplastic syndromes (MDS) remains controversial. We studied the expression of CD95 and CD95L in CD34+ cells of patients with newly diagnosed MDS by flow cytometry, and examined the relation between this expression and FAB and WHO types, total number of CD34+ bone marrow (BM) cells and the degree of peripheral cytopenias. The patients with refractory anemia (RA) and sideroblastic anemia showed CD34+ cells in numbers comparable to normal donors, but had a higher percentage of CD95/CD34 and CD95L/CD34 positive cells. An inverse correlation was found between these cells and the total CD34+ cells. This was also observed when only patients with RA were analyzed. In RAEB, however, CD95/CD95L expression correlated with CD34+ cells but not with the percentage of BM blasts. After classification by the WHO proposal, the patients with refractory cytopenias with multilineage dysplasia showed features intermediary between RA and RAEB. No significant correlation was seen between the expression of CD95/CD95L and the peripheral blood counts. These results are in keeping with the hypothesis that, as the number of MDS precursors increases during disease progression they become less succeptible to apoptosis.

Research Articles| March 10 2003 Polymorphisms of Glutathione S-Transferase Mu1 (GSTM1) and Theta1 (GSTT1) Genes in Multiple Myeloma Subject Area: Hematology , Oncology Manoela M. Ortega; Manoela M. Ortega aDepartment of Internal Medicine, Search for other works by this author on: This Site PubMed Google Scholar Helvia Nascimento; Helvia Nascimento aDepartment of Internal Medicine, Search for other works by this author on: This Site PubMed Google Scholar Monica B. Melo; Monica B. Melo bHaematology and Haemotherapy Centre, State University of Campinas, Campinas, São Paulo, Brazil Search for other works by this author on: This Site PubMed Google Scholar Maria T. Teori; Maria T. Teori bHaematology and Haemotherapy Centre, State University of Campinas, Campinas, São Paulo, Brazil Search for other works by this author on: This Site PubMed Google Scholar Fernando F. Costa; Fernando F. Costa aDepartment of Internal Medicine, Search for other works by this author on: This Site PubMed Google Scholar Carmen S.P. Lima Carmen S.P. Lima bHaematology and Haemotherapy Centre, State University of Campinas, Campinas, São Paulo, Brazil Search for other works by this author on: This Site PubMed Google Scholar Acta Haematol (2003) 109 (2): 108–109. https://doi.org/10.1159/000068486 Article history Received: July 18 2002 Accepted: July 23 2002 Published Online: March 10 2003 Content Tools Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Facebook Twitter LinkedIn Email Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Manoela M. Ortega, Helvia Nascimento, Monica B. Melo, Maria T. Teori, Fernando F. Costa, Carmen S.P. Lima; Polymorphisms of Glutathione S-Transferase Mu1 (GSTM1) and Theta1 (GSTT1) Genes in Multiple Myeloma. Acta Haematol 1 March 2003; 109 (2): 108–109. https://doi.org/10.1159/000068486 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search Dropdown Menu toolbar search search input Search input auto suggest filter your search All ContentAll JournalsActa Haematologica Search Advanced Search Article PDF first page preview Close Modal This content is only available via PDF. 2003Copyright / Drug Dosage / DisclaimerCopyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. You do not currently have access to this content.

Hemoglobin F (HbF) is an effective inhibitor of HbS polymerization. Hydroxyurea (HU) is used to increase HbF synthesis and improve the clinical course of sickle cell disease (SCD) patients. We studied a series of laboratory parameters concerning HbF production and reticulocyte response, and compared data between two groups: 1) 13 SCD patients treated with HU, and 2) 33 untreated SCD patients. Higher values of Hb concentration, mean cell volume (MCV), mean cell hemoglobin (MCH), mean reticulocyte volume (MRV), HbF concentration, percentage of F-cells, and amount of HbF/F-cells were observed in the treated group of patients. There was no correlation between Hb and HbF elevations. The reticulocyte count, immature reticulocyte count, mean fluorescence index (MFI), and neutrophil count were significantly lower in treated patients. Taken together, these findings suggest that a decreased hemolytic process occurred in patients undergoing HU treatment. There was a significant correlation between MCV and HbF, between MRV and HbF, and between MRV and F-cell in patients taking HU. These data indicate that macroreticulocytes correspond to F-reticulocytes, and that an increase in MRV in SCD patients using HU may be an indirect signal of F-cell production. The concentration of HbF/F-cells was higher in patients treated with HU, but this increase apparently was independent of F-cell production. Reticulocyte (RTC) parameters, as assessed by hematological analyzers, may be useful for following erythropoietic changes in patients receiving HU, and can indirectly indicate HbF and F-cell production induced by HU therapy.

BACKGROUND:Clinical studies have shown that amifostine (AMF) improves peripheral cytopenias in myelodysplastic syndromes (MDS). We studied the expression of Fas/FasL on CD34+ cells in low risk MDS and its change after AMF treatment.PATIENTS AND METHODS:Patients received AMF 400 mg/m2 3x/week for 3 weeks and 2 weeks off treatment. Peripheral blood counts and BM cytology were analysed before and after 2 courses. Quantification of CD34+ cells and CD34/CD95 and CD34/CD95L ones were performed by flow cytometry.RESULTS:Seventeen patients were treated. After 2 months, 8 patients showed a rise in neutrophil count. Hemoglobin increased in 1 and thrombocytes in 2 patients. Before treatment, responding patients presented a significantly lower expression of Fas (median 53%) and FasL (median 26%) than non-responders (85% and 70%, respectively). BM lymphocytes were significantly lower in responders (median 14.5% and 27.4%, respectively). In responders CD34+ cells decreased after treatment. The change in neutrophil count after treatment presented an inverse correlation with the percentage of BM lymphocytes before treatment (r = 0.0-0.58; p = 0.02).CONCLUSION:Response to AMF may be influenced by the intensity of the immune reaction in BM.

Aim: To test the usefulness of a random urine specimen albumin to creatinine ratio (A/C) in predicting 12 hour urinary albumin excretion (12UA) in patients with sickle cell disease. Methods: 12UA and A/C were measured in nocturnal urine collections and random morning urine samples, respectively, of 72 patients with sickle cell disease. Results: The correlation of A/C values with 12UA values did not provide support for the use of random urine specimens for predicting urinary albumin excretion (UAE) in these patients. However, values of A/C ≥ 0.45 and < 0.45 were indicative of raised and normal UAE, respectively. The sensitivity, specificity, and accuracy of the test were 100.0%, 87.2%, and 91.7%, respectively. Conclusions: This method cannot be recommended for predicting 12UA in patients with sickle cell disease, but it is useful for selecting patients who should collect 12 hour urine for the estimation of UAE.