ABSTRACT
Objective: to assess the frequency of aberrant antigens expression in acute leukemias and their possible prognostic significance in a group of Jordanian patients
Methods: a retrospective study of acute leukemia cases was conducted at King Hussein Medical Centre over 3 years [January 2012 to December 2014]. A total of 368 cases of acute leukemia diagnosed by multi parameter flow cytometry performed on peripheral blood and/ or fresh bone marrow aspirates. The co-expression of myeloid markers on lymphoblasts and lymphoid markers on myeloblasts was analyzed. The findings were correlated with remission status
Results: 368 cases of acute leukemias were retrieved; these were: 192 [52%] cases of acute myeloid leukemia [AML], 173 cases [47%] of acute lymphoblastic leukemia [ALL] and 3 cases [1%] with mixed phenotype acute leukemia. Aberrant immunophenotype expression was observed in 44 [23%] AML cases and in 50 [29%] of ALL cases. CD7 was the commonest aberrant lymphoid marker expressed in AML which was noted in 19/44 [43%]. Of the aberrant B-ALL cases, CD33 were expressed in 18/38 [47%] and CD13 in 14/38 [37%]. 212 out of 368 cases [58%] were followed up in our centre during treatment program and stratified into remission and non-remission groups based on morphologic assessment of peripheral blood and bone marrow aspirates. These tests were carried out at day 21 of induction therapy, completion of treatment and any clinical deterioration during the study period. 70% of non-remission ALL and 53% of non-remission AML had aberrant phenotypes. No significant differences were noted between classical and aberrant acute leukemias regarding age, sex and blasts count
Conclusion: the incidence of aberrant antigen expression in acute leukemia was comparable with most published international data. Such aberrant antigen expression may represent a poor prognostic indicator among this group of Jordanian patients. These findings may help to recognize patients with high risk group and low remission rate. Further studies are needed to confirm the correlation between aberrant phenotypes with prognosis and therapeutic response of acute leukemia
ABSTRACT
To analyze the serum protein electrophoresis and immunofixation results in a group of patients confirmed to have plasma cell myeloma by bone marrow findings. The study was conducted at Princess Iman Center for Research and Laboratory Sciences at King Hussein Medical Center. A total of 30 patients were studied in the time period between June 2013 and December 2013. For each patient serum protein electrophoresis and immunofixation were performed. The bone marrow aspirate and biopsy results were reviewed. The patterns of serum protein electrophoresis were analyzed and confirmed by immunofixation electrophoresis. All the patients' serum protein electrophoresis results showed the presence of monoclonal band. No non-secretory myeloma cases were found. The location of bands was in gamma or beta regions; 23[76.6%] of 30 were found in gamma region, whereas 7[23.4%] in beta region. The largest proportion of IgG paraprotein type was found in gamma region and majority of IgA and IgM types were found in beta region. The most common paraprotein isotype found in beta region was IgA with frequency of 71.4%, 14.3% for IgG and 14.3% for IgM. The most frequent paraprotein [heavy chain] isotype seen was as follows: 80%, 16.6%, and 3.3% for IgG, IgA, and IgM respectively. Regarding light chains, kappa light chain was the most frequent [60%].The patterns of serum protein electrophoresis and immunofixation of this group of plasma cell myeloma patients showed the presence of M-band in all patients. For early diagnosis patients suspected to have plasma cell myeloma the serum protein electrophoresis and immunofixation remain an easy gold standard test for cases with secretory plasma cell myeloma
ABSTRACT
To assess the matching between bone marrow aspirate and bone marrow biopsy findings done simultaneously in patients with clinical indications of bone marrow examination, to stand on the diagnostic efficacy of each in various bone marrow diseases. This study was conducted at King Hussein Medical Center, Haematology and Oncology Department in collaboration with Hematopathology Division of the Laboratory Department. The Pathology reports of both bone marrow aspirate and biopsy done simultaneously on 500 cases in the period between January and December 2012 were retrospectively reviewed. About 76.2% of the cases showed positive correlation between the bone marrow Aspirate and biopsy findings. The highest correlation was noted in diffuse bone marrow diseases, and hematological malignancies; including acute myeloid leukemia [92.8%], idiopathic thrombocytopenic purpura [91%], and chronic myeloid leukemia [90.9%], poor correlation was observed in infiltrative bone marrow diseases including idiopathic myelofibrosis was 0%, lymphoma involvement 14.2%, and bone marrow metastasis 18.2%. The diagnostic accuracy was 76.2% for aspirates and 98.8% for biopsy. This study shows that bone marrow aspirate is of diagnostic value mostly in diffuse bone marrow diseases and is of limited value in infiltrative bone marrow diseases where biopsy is mandatory. In general, there was a good correlation between the aspirate and biopsy findings
ABSTRACT
To study the immunophenotypic profile of acute leukemia cases, using multicolor flow cytometry for lineage subtyping. This is a retrospective review of acute leukemia cases conducted at department of Hematopathology at King Hussein Medical Center between January 2011 to December 2013. A total of 340 acute leukemia cases were analyzed using flow cytometry method. The diagnosis was based on morphological assessment of peripheral blood and bone marrow aspirate smears and immunophenotyping by flow cytometry. A total of 340 cases of acute leukemia were studied. 164 cases [48.2%] were acute lymphoblastic leukemia, 176 [51.8%] were acute myeloid leukemia. Acute leukemia was diagnosed among adults in 51.8% whereas 48.2% were children. Of the acute lymphoblastic leukemia cases, 130 cases [79.3%] were B-cell type and 34 cases [20.7%] were T-cell type. All cases of B-acute lymphoblastic leukemia showed expression of pan B-cell markers [CD19, CD22 and cytoplasmic CD79a] and 117 [90%] of cases expressed CD10. Cytoplasmic CD3 and CD5 were the most sensitive markers for diagnosis of T-acute lymphoblastic leukemia. Of the 176 cases of acute myeloid leukemia, 16 cases [9%] were identified as acute promyelocytic leukemia, while the rest 160 cases showed expression of CD34 and HLA-DR in 41.4% and 68.7% retrospectively. None of the cases of acute promyelocytic leukemia were positive for both CD34 and HLA-DR. CD13 and CD33 were expressed in all cases of acute myeloid leukemia studied. Flow cytometric immunophenotyping is a powerful method for accurate diagnosis, identification and subtyping of acute leukemia. Furthermore, it has a great therapeutic and prognostic implications on such cases with unique usefulness in differentiation between acute lymphoblastic leukemia and acute myeloid leukemia-M0. Immunophenotyping results of acute leukemia in this group of Jordanian patients were comparable to the international data. By combining morphology and immunophenotyping, we were able to diagnose and classify cases of acute leukemia at our center where peripheral blood and adequate bone marrow aspirates are available
Subject(s)
Adult , Adolescent , Child , Female , Humans , Male , Immunophenotyping , Flow Cytometry , Retrospective Studies , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Leukemia, Myeloid, AcuteABSTRACT
To determine the spectrum of cytogenetic abnormalities of acute lymphoblastic leukemia in children and adults at King Hussein Medical Center. A retrospective review of raw bone marrow aspirate cytogenetic analysis reports was conducted at Princess Iman Research and Laboratory Sciences Center at King Hussein Medical Center during the period between Jan 2010 and Apr 2014. A total of 97 patients were studied regarding: age, gender, and cytogenetic analysis. The age was categorized into two groups [14 years as adult group]. Descriptive analysis using frequencies was used to describe the study variables. Fifty-two [53.6%] cases were males and 45 [46.4%] were females. Their ages ranged between six months and 72 years. A total of 72 [74.2%] patients were children and 25 [25.8%] patients were > 14 years old. Of all pediatric acute lymphoblastic leukemia cases, 52.8% [38 cases] were negative for all the cytogenetic abnormalities, while 47.2% [34 cases] revealed one or more cytogenetic abnormalities. Translocation t[12;21], hyperdiploidy [>50 chromosomes or DNA index >1.16] were the predominant cytogenetic abnormalities in children with lymphoblastic leukemia. In the adult lymphoblastic leukemia group, 68.0% [17 cases] were negative for all the cytogenetic abnormalities, while 32% [8 cases] had cytogenetic abnormalities. Hyperdiploidy was the most common [20.0%, 5 patients] followed by translocation t [9;22] [8.0%, 2 patients]. Distribution and patterns of chromosomal abnormalities of lymphoblastic leukemia differ between children and adults. Translocation t[12;21], hyperdiploidy and rearrangements / translocations involving the MLL gene at chromosome 11q23 were the most commonly encountered in children. Hyperdiploidy was prevalent in adults, while no adult cases with 11q23 rearrangements or t[12;21] were encountered
Subject(s)
Adult , Adolescent , Aged , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Cytogenetics , Prevalence , Retrospective Studies , Bone Marrow ExaminationABSTRACT
To identify the importance of flow cytometry [FCM] in diagnosis and subclassifying acute lymphoblastic leukemia, and to highlight its capability to detect antigen aberration. The Results of flow cytometry for 165 patients, between January 2006 and December 2011 who were diagnosed with acute lymphoblastic leukemia [ALL] were retrospectively reviewed with respect to age and gender distribution and immunophenotypic findings. 63% of patients were children [104 out of 165 patients] with age less than fourteen years old. 114 patients were male while 51 patients were female with male to female ratio 2.2: 1. Precursor-B- acute lymphoblastic leukemia represents eighty percent [132 patients] of cases, of 106 patients [87.6%] were [CD10/CD19] positive, 125 patients [94.7%] were positive for cytoplasmic CD79a, 126/129 [97.6%] were positive for HLA-DR, and 15 patients [11.36%] were CD10 negative. Aberrant myeloid antigen expression was noted; CD33 and CD13 were positive in 15/113 [13.3%] and 2/108 [1.85%] respectively. On the other hand precursor-T- acute lymphoblastic leukemias were found in thirty three patients, 84.4% of them were Anti-TdT positive, and all were negative for B-cell markers. Myeloid antigen expression results were as follows; 1/29 [3.4%] and 2/29 [6.9%] positive for CD33 and CD 13 respectively. Flow cytometry is a golden tool in diagnosis and identifying ALL subtypes. Precursor-B- acute lymphoblastic leukemia represents most of ALL cases with minority of cases are CDlOnegative. Aberrant myeloid antigen expression would not change the diagnosis of ALL in either B- or T- subtypes. Further clinical correlation is needed to figure out aberrant markers prognostic implications
ABSTRACT
Kidneys are the major source of erythropoietin production, anemia of chronic kidney disease is mainly caused by erythropoietin deficiency. Chronic kidney disease patients are treated with erythropoiesis stimulating agents [epoietin Alfa, epoietin beta], which is considered the gold standard for treatment of anemia in chronic kidney disease patients. One of rare and serious complication in patients treated by erythropoiesis stimulating agents is pure red cell aplasia, which is characterized by rapid decline in hemoglobin concentration of 5-10g/l per week, with normal count of white blood cells and platelets, and absolute reticulocyte count less than 10,000/ml. Pure red cell aplasia is treated by stopping erythropoiesis stimulating agents, cytotoxic drugs and peginesatide
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To describe the hematological findings among children with celiac disease on presentation. This is a retrospective review which was conducted in the Pediatric Gastroenterology Department at Queen Rania Al Abdullah Hospital for Children. The records of children with celiac disease were reviewed between January 2006 and December 2012. The age of children included in the study was less or equal to 14 years on diagnosis. Complete blood count, serum ferritin, folate, vitamin B12, prothrombin time, partial prothrombin time, international normalized ratio, and tissue glutaminase antibody [IgA and IgG] were performed for all patients prior to the diagnosis. Upper gastrointestinal endoscopy was performed for all children included in the study and multiple duodenal biopsies samples were obtained during the procedure for routine histological analysis. A total of 111 children were included in the study; 53 [47.7%] were males and 58 [52.3%] were females. The mean age at diagnosis was 9 years. All children had positive tissue glutaminase antibody IgA, IgG, or both. Eleven children had leukopenia, 13 had lymphopenia, two had neutropenia, while eight had eosinophilia. Thirty four [30.4%] children had anemia. Twenty eight [25.2%] children had serum ferritin less than 7ng/ml, 30 [27%] had serum folate less than 5ng/ml and 9 [8.1%] children had vitamin B12 less than 200 pg/ml. Seventeen [60.7%] children who had serum ferritin less than 7ng/ml had also low serum folate [p=0.001]. Four [36.4%] out of eleven children with leukopenia had serum folate below 5ng/dl [p=0.2]. Vitamin B12 level in children with absolute lymphocyte count less than 1500/microL was significantly less than that of children with equal or more than 1500/microL [P value 0.02]. Twelve [10.8%] children had thrombocytosis. The mean hemoglobin level and serum ferritin were significantly lower in children with thrombocytosis than those with normal platelets [p<0.01]Celiac disease is associated with a diversity of hematological findings that include leukopenia, lymphopenia, neutropenia, and eosinophilia, as well as anemia, thrombocytopenia and thrombocytosis
ABSTRACT
We report on a 28 year old female who was diagnosed as a case of Behcet's syndrome and referred to our rheumatology clinic for further evaluation regarding unexplained fever and leukocytosis. Blood film revealed anemia and persistent eosinophilia. Bone marrow examination showed eosinophilic leukemia which is a rare condition especially in the female gender. Although Behcet syndrome can be associated with eosinophilia, the clinical picture was suggestive of myeloid neoplasm
ABSTRACT
A 31-year-old male patient presented with fever and pancytopenia. He was diagnosed as a case of chronic anemia since early childhood. The etiology of the anemia was not known. The patient was transfusion dependent, and he had been maintained on erythropoietin for three years prior to admission. A bone marrow examination revealed prominent proliferation of immature and dysplastic erythroid precursors. Acute erythroid leukemia of the pure erythroid subtype was suspected. However, because of the history of erythropoietin therapy a definite diagnosis was not made. On follow-up one month later, the marrow changes had reversed to normal.
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To determine the spectrum of various types of malignant lymphoma in children and adults at King Hussein Medical Center according to the World Health Organization classification 2001. A retrospective review of the histopathological subtypes of all primary lymphoma cases was conducted at Princess Iman Research and Laboratory Sciences Center at King Hussein Medical Center during the period between January 2004 and December 2008. A total of 485 patients were studied regarding: age, gender, and Lymphoma type using the World Health Organization classification of lymphoid neoplasms. Descriptive analysis using frequencies was used to describe the study variables. Two hundred seventy-four [56.5%] cases were males and 211 [43.5%] were females. Their ages ranged between two and ninety years. A total of 61 [12.6%] patients were children and 424 [87.4%] patients were > 14 years old. Of 485 patients included in the study, 342 patients [70.5%] had non-Hodgkin's lymphoma and 143[29.5%] had Hodgkin's lymphoma. Two hundred twenty nine [47.2%] of affected patients aged 50 years and above, non-Hodgkin's Lymphoma accounted for 206 patients [90%] of them. Of all pediatric lymphoma cases, Hodgkin's lymphoma accounted for 41% [25 cases] and Non-Hodgkin's Lymphoma 59% [36 cases]. Burkitt's lymphoma was the predominant lymphoma in children. In the adult NHL group, diffuse large B-cell lymphoma was the most common followed by follicular lymphoma. In the HL group, the nodular sclerosis variant was the most frequent [63.6%, 91 patient] followed by the mixed cellularity type [20.3%, 29 patient]. Distribution and patterns of lymphoma differs between children and adults. Diffuse large B-cell lymphoma is the most commonly encountered lymphoma in adults. Burkitt's lymphoma and Hodgkin's lymphoma are the predominant childhood lymphomas.
Subject(s)
Humans , Male , Female , Lymphoma, B-Cell/pathology , Lymphoma, B-Cell/epidemiology , Brain Neoplasms/pathology , Retrospective Studies , Review Literature as TopicABSTRACT
To highlight some of the significant applications of flow cytometric immunophenotyping in the diagnosis of Primary Immunodeficiency Disease. We reviewed the medical records of 135 consecutive patients who were referred to the Immunology Clinic at King Hussein Medical Center with a flow cytometry based diagnosis of Primary Immunodeficiency Disease between January 2000 to August 2009. The medical records of fl5 patients with history of recurrent or persistent infections were reviewed. Seventy seven [57%] patients were males and 58[43%] were females. They aged between 2 and 120 months with a mean age of 13 months. Flow cytomerty-based diagnosis was identified in 68 [50.3%] patients. Predominant antibody deficiency was diagnosed in 114 [10.3%] patients. There were 35[26%] patients with T and B cell immunodeficiency. There were 6 patients' satisfied diagnostic criteria of possible HyperlgM lmmunodeficiency syndrome. Diagnosis of severe combined immunodeficiency was retrieved in 22[16.2%] patients. Primary phagocytic disorder was the diagnosis in 34 [25%] patients. Dihydrorhodamine flow cytomerty-based burst test was confirmatory for Chronic Granulomatous Diseases in one patient while in the other 14 patients diagnosis was based on nitroblue tetrazoleoum test and genetic mutation study. There were 8 [6%] patients with other well defined immunodeficiency syndromes; one patient with Wiskott Aldrich Syndrome, 5 patients with Ataxia Telangectasia, one with Bloom syndrome, and one with DiGeorge anomaly. Eight [6%] patients were found to have an immunedysregulation syndrome. There were 8[6%] patients with an undefined primary immunodeficiency. Post Bone marrow transplantation Immunereconstitution of T-, B-cells and Leukocyte adhesion molecules were identified in 14 patients with appropriate Flow cytomerty immunophenotyping assay. Flow cytometric immunophenotyping of leucocytes appears to be an efficient and rapid tool in the diagnosis and follow-up of immunodeficient patients, supporting early recognition, which is reflected on reduced morbidity and improved survival
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The aim of this study was to define the spectrum of alpha-thalassemia determinants existing in Jordan. A total 286 suspected alpha-thalassemia subjects including 29 hemoglobin Hb [Hb H] patients were examined by polymerase chain reaction and restriction enzyme digestion. Polymerase chain reaction product was examined by agarose gel electrophoresis. Five different alpha-thalassemia determinants were characterized in 336 chromosomes. The most prevalent alpha -thalassemia determinant was the single gene deletion -alpha[3.7] [45%]. The non-gene deletion alpha [5nt] accounted for 27% of thalassemic chromosomes, followed by the non-gene deletional determinant alpha [T-Saudi][23%]. The two-gene deletional determinant -[MED] was characterized only in 4% of thalassemic chromosomes. Triplicated alpha -gene determinant was observed in two heterozygous individuals [alpha alpha alpha / alpha alpha]. Four different genotypes were found to be responsible for Hb H disease. Homozygosity for the non-deletional determinant alpha[T-Saudi] [alpha T-Saudi] alpha/alpha T-Saudi]alpha] was observed in the majority of those patients [76%] and was found to be associated with high Hb H levels. Less commonly, Hb H disease occurred as a result of compound hterozygosity between -[MED] determinant with other determinants; [-[MED] /alpha [T-Saudi] alpha] [--MED]/ alpha[5nt] alpha], [--[MED]/ -alpha[37] alpha]. The outcome of this pilot study provides valuable and basic information, about the spectrum of a-thalassemia mutations in Jordan that might be useful in setting a strategy for molecular diagnosis of alpha-thalassemia carrier status and Hb H disease in this country