ACQUIRED β−THALASSEMIA IN CHILDREN WITH ACUTE LYMPHOBLASTIC LEUKEMIA (ALL)

Thalassemias are heterogeneous group of genetic disorders. β-thalassemia is existed due to impaired production of beta globins chains, which leads to a relative excess of alpha globin chains. The abnormalities of haemoglobin synthesis are usually inherited but may also arise as a secondary manifestation of another disease, most commonly haematological neoplasia. This article presenting two cases of acquired β-thalassemia in children with ALL focusing on the diagnosis and the possible relationship between the two haematological diseases. The first case is a four (4) year old boy with ALL-L1 type at maintenance phase of chemotherapy, he suffered from anaemia with Hb 8.0 g/dL, WBC 22,600/mm3 and platelets count of 200,000/mm3, peripheral blood smear revealed anisocytosis, polychromes, hypochromia, basophilic stippling, and normoblastocytes. The result of Hb electrophoresis of Hb A of 54.9%, Hb F of 29.4%, Hb E of 13.4% and Hb A2 of 2.3%. The patient was diagnosed as ALL-L1 type and β-thalassemia. The second case, is a 13 year old girl with remission ALL-L1 type after chemotherapy, she suffered from anaemia with Hb 6.7 g/dL, WBC 12,400/mm3, platelet count was 200,000/mm3, and peripheral blood smear obtained anisocytosis, hypochromia, normoblastocytes, myelocytes and basophilic stippling. The result of Hb electrophoresis are: Hb F 0.41%, Hb A1c 0.78%, Hb A2 2.95% with the conclusion of a β-thalassemia trait, this patient was diagnosed with ALL-L1 type remission + β-thalassemia trait. The case reviewers assume that acquired β-thalassemia which happened in those patients were the altered expression of globin chain which mechanism for this syndrome might be the acquisition of a mutation that affects RNA or proteins involved in β-globin gene regulation and resulting the reduction of the (α/β)-globin biosynthetic ratios, or/and associated with chemotherapy-inducement.

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A CLINICOPATHOLOGICAL STUDY OF ACUTE AND CHRONIC LEUKEMIA IN A TERTIARY CARE HOSPITAL

10.36106/ijar/7402428 ◽

2021 ◽

pp. 72-74

Author(s):

Sarat Das ◽

Prasanta Kr. Baruah ◽

Sandeep Khakhlari ◽

Gautam Boro

Keyword(s):

Acute Myeloid Leukemia ◽

Acute Lymphoblastic Leukemia ◽

Peripheral Blood ◽

Myeloid Leukemia ◽

Blood Smear ◽

Lymphoblastic Leukemia ◽

Peripheral Blood Smear ◽

Care Hospital ◽

Cytochemical Study ◽

Acute Myeloid

Introduction: Leukemias are neoplastic proliferations of haematopoietic stem cells and form a major proportion of haematopoietic neoplasms that are diagnosed worldwide. Typing of leukemia is essential for effective therapy because prognosis and survival rate are different for each type and sub-type Aims: this study was carried out to determine the frequency of acute and chronic leukemias and to evaluate their clinicopathological features. Methods: It was a hospital based cross sectional study of 60 patients carried out in the department of Pathology, JMCH, Assam over a period of one year between February 2018 and January 2019. Diagnosis was based on peripheral blood count, peripheral blood smear and bone marrow examination (as on when available marrow sample) for morphology along with cytochemical study whenever possible. Results: In the present study, commonest leukemia was Acute myeloid leukemia (AML, 50%) followed by Acute lymphoblastic leukemia (ALL 26.6%), chronic myeloid leukemia (CML, 16.7%) and chronic lymphocytic leukemia (CLL, 6.7%). Out of total 60 cases, 36 were male and 24 were female with Male:Female ratio of 1.5:1. Acute lymphoblastic leukemia was the most common type of leukemia in the children and adolescents. Acute Myeloid leukemia was more prevalent in adults. Peripheral blood smear and bone Conclusion: marrow aspiration study still remains the important tool along with cytochemistry, immunophenotyping and cytogenetic study in the diagnosis and management of leukemia.

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Clinical Characteristics of Patients with a RBC Fragment Flag on the Advia® 2120 Automated Hematology Analyzer.

Blood ◽

10.1182/blood.v110.11.5153.5153 ◽

2007 ◽

Vol 110 (11) ◽

pp. 5153-5153

Author(s):

Jonathan Ben-Ezra

Keyword(s):

Iron Deficiency ◽

Iron Deficiency Anemia ◽

Peripheral Blood ◽

Blood Smear ◽

Lymphoblastic Leukemia ◽

Peripheral Blood Smear ◽

Deficiency Anemia ◽

Clinical Criteria ◽

Hematology Analyzer ◽

Automated Hematology Analyzer

The clinical diagnosis of thrombotic thrombocytopenia purpura (TTP) is a difficult one to make. It is based on clinical criteria, one of which is a microangiopathic hemolytic anemia, characterized morphologically by the presence of schistocytes on the peripheral blood smear. The ADVIA 2120 automated hematology analyzer is able to quantify the presence of red blood cell (RBC) fragments. We studied the ability of the ADVIA 2120 to be able to detect RBC fragments in the blood of TTP patients, and the characteristics of all patients in whom RBC fragments were obtained. During the study period, 6 TTP patients were studied. The initial numbers of RBC fragments ranged from 0.02–0.05 × 106 cells/μl. During the course of plasmapheresis, these numbers decreased to 0.00–0.02 × 106 cells/μl, corresponding to a rise in the platelet count. Figure Figure In the course of a month, 52 blood samples on 39 patients were flagged by the hematology analyzer to have RBC fragments (0.01–0.12 × 106 cells/μl). 52 Samples with RBC Fragment Flag Hemoglobin Platelets RDW Range 4– 14.3 g/dl 5–906 × 103/ul 13.9– 28.6% Number Abnormal 46 (<13.0 g/dl) 23 (<160 × 103/ul) 51 (>14.1%) Within this population, there were two patients with TTP, and one with DIC. Four of the samples did not have detectable schistocytes upon visual inspection of the peripheral blood smear. There were 19 samples from 14 patients who had RBC fragment counts ≥ 0.04 × 106 cells/μl. 19 Specimens with RBC Fragments ≥ 0.04 × 106/ul Hemoglobin Platelets RDW Range 8– 14.1 g/dl 59– 906 × 103/ul 16.4– 25.3% Number Abnormal 15 (<13 g/dl) 4 (<160 × 103/ul) 19 (>14.1%) The diagnoses in these 14 patients were iron deficiency anemia (4 patients), thalassemia trait (2), acute lymphoblastic leukemia (2), and one each with TTP, sickle cell anemia, heart failure, kidney stone, cerebrovascular accident (CVA), and end stage renal disease. We conclude that the RBC fragment flag on the ADVIA 2120 is nonspecific. Although it does detect schistocytes in TTP, these are often present in low numbers. Quantitatively, the most numerous RBC fragments are found in diseases with marked anisopoikilocytosis, such as iron deficiency anemia.

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The first case report of neuroacanthocytosis in Thailand: Utilization of a peripheral blood smear technique for detecting acanthocytes

Clinical Neurology and Neurosurgery ◽

10.1016/j.clineuro.2010.03.027 ◽

2010 ◽

Vol 112 (6) ◽

pp. 541-543 ◽

Cited By ~ 3

Author(s):

Natlada Kanjanasut ◽

Priya Jagota ◽

Roongroj Bhidayasiri

Keyword(s):

Case Report ◽

Peripheral Blood ◽

Blood Smear ◽

Peripheral Blood Smear ◽

First Case

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Nietypowy obraz hemoglobinopatii współistniejącej z talasemią β

Nowa Pediatria ◽

10.25121/np.2018.22.4.145 ◽

2018 ◽

Vol 22 (4) ◽

Author(s):

Anna Adamowicz-Salach ◽

Beata Burzyńska ◽

Alicja Siwicka ◽

Katarzyna Smalisz ◽

Michał Matysiak

Keyword(s):

Blood Smear ◽

Globin Gene ◽

Gene Clusters ◽

Peripheral Blood Smear ◽

Target Cells ◽

Genetic Tests ◽

Autosomal Recessive Disorders ◽

Hemoglobin Synthesis ◽

First Case ◽

Diagnostic Panel

Introduction. Inherited hemoglobin disorders, including thalassemia and hemoglobinopathies, are frequently occurring diseases in the world. Thalassemias are autosomal recessive disorders of hemoglobin synthesis. Underlying molecular defects in the α-globin or β-globin gene clusters form the basis of defective hemoglobin synthesis and the various inherited forms of α- halasemias or β-thalasemias. Thalassemia is diagnosted where hemoglobin levels, volume of red blood cells (MCV) and mean cellular hemoglobin (MCH) are significantly reduced. Aim. We want to introduce atypical clinical falieure of hemoglobinopathy. Material and methods. We presented two children with anemia. In first case it was a boy with normal MCV and reduced MCH, next case it was a girl after splenectomy with increased MCV and MCH. In both cases the red cells were hypochromic and also target cells were present in peripheral blood smear. Hemoglobin electrophoresis, measurement of HbA2 and HbF levels were performed. Results. Test results were characteristic for thalasemia β. Extending the diagnostic panel with genetic tests helped to identify in both of children thalassemia α silience carrier (-α/αα). In first case unstabile hemoglobin Hb Köln (Codon 98(295G > A)) was also detected and in second unstabile hemoglobin Hb Bruxelles (deletions del 42/β+). Conclusion. Only the genetic tests made it possible to explain atypical blood smear for thalassemia.

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