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Myelodysplastic Syndromes
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Blood, apart from anemia, which is the most common finding in MDS, may show neutropenia and/or thrombocytopenia. Red blood cells are most often macrocytic, but may be normocytic (microcytic anemia is unusual for MDS). The MDS category RARS is usually associated with mean corpuscular volume (MCV) values between 100 and 105. The red cells often display anisopoikilocytosis and may show basophilic stippling. Poikilocytosis includes dacrocytes (teardropshaped cells), acanthocytes (spur-like cells), and oval macrocytes. Granulocytes are often hypogranular and hypolobated. Hypolobated granulocytes are reminiscent of inborn Pelger–Huët anomaly and are therefore called pseudo-Pelger cells. Platelets may also display atypia (including giant forms) when examined on oil magnification. Blasts, nucleated red blood cells, basophils, and micromegakaryocytes may be present on blood smear. MDS with basophilia was reported to be associated with worse outcome. Presence of 2%–4% blasts in the blood is diagnostic for RAEB-1 (even if the number of blasts in the BM is <5%) and 5%–19% for RAEB-2. Based on the WHO classification, the presence of Auer rods is diagnostic for RAEB-2, even if the number of blasts in the blood is <5%.
Genotype-Phenotype Correlation of β-Thalassemia in Malaysian Population: Toward Effective Genetic Counseling
Published in Hemoglobin, 2020
Uday Y. H. Abdullah, Hishamshah M. Ibrahim, Noraesah Binti Mahmud, Mohamad Zaki Salleh, Lay Kek Teh, Mohd Nur Fakhruzzaman bin Noorizhab, Bin Alwi Zilfalil, Haitham Muhammed Jassim, Prapin Wilairat, Suthat Fucharoen
The significantly lower mean MCV and MCH values in the patient groups as compared with the values in the healthy blood donors (p = 0.001), indicating ineffective erythropoiesis in patients group compared with normal erythropoiesis in healthy blood donors. However, the values in patients with Hb E/β-thal and β-TM showed no significant difference (p = 0.837) (Table 3). This is due to the same level of suppression of endogenous erythropoiesis caused by the transfusion program in the management of these two types of thalassemia. The significantly higher RDW-CV values in patient groups compared to its value in the healthy blood donors (p = 0.001) indicate the more severe degree of anisopoikilocytosis in β-thal secondary to the effects of ineffective and dyserythropoiesis [25].
Severe acute kidney injury owing to rhabdomyolysis and intravascular haemolysis in an 11-year-old child with G6PD deficiency
Published in Paediatrics and International Child Health, 2019
Milan Talwar, Sriram Krishnamurthy, Narayanan Parameswaran, C. G. Delhikumar, Satish Haridasan, Bheemanathi Hanuman Srinivas
Haemoglobin was 3.3 g/dL, total leucocyte count 3.3 × 109/L and platelet count 260 × 109/L. Red blood cells were normocytic and normochromic with moderate anisopoikilocytosis. Blood film demonstrated many fragmented red blood cells and reticulocytosis (corrected reticulocyte count 5%). Blood urea was 68.2 mmol/L (2.9–8.2), serum creatinine 610 μmol/L (53–106), sodium 136 mmol/L and potassium 4.1 mmol/L. Urinalysis showed no RBCs but tested positive for blood by multistix on several occasions. Lactate dehydrogenase was 114.7 μkat/L (1.7–3.4) and creatinine kinase total (CPK) 136 μkat/L (0.67–2.5). The investigations were consistent with severe intravascular haemolysis in association with rhabdomyolysis, resulting in haemoglobinuria and myoglobinuria. Rhabdomyolysis was diagnosed on the basis of high levels of LDH, elevated blood CPK and the presence of myoglobin casts in the renal tubules demonstrated in the renal biopsy on immunohistochemistry.
Unstable Hemoglobin Variants: The Need for Clinical Vigilance in Infants with Congenital Jaundice
Published in Hemoglobin, 2019
Hua Jiang, Jian-Ying Zhou, Jian Li, Dong-Zhi Li
On this referral, physical examination showed palpable spleen and liver below the costal margin and distinct jaundice without other notable findings. Liver function tests showed enhanced levels of aspartate transaminase (AST) (553.0 U/L), serum bilirubin (61.0 μmol/L) and indirect bilirubin (52.3 μmol/L). Hepatitis markers were negative. Red cell morphology showed marked anisopoikilocytosis. Heinz bodies were found in about 10.0% of the erythrocytes in peripheral blood smears after overnight incubation at 37 °C with supravital staining. Hematological investigations revealed the following: RBC 3.65 × 1012/L, mean corpuscular volume (MCV) 95.6 fL, mean corpuscular Hb (MCH) 29.3 pg, Hb 10.7 g/dL and reticulocyte count of 12.2%. Hemoglobin analysis by capillary electrophoresis (CE) (CapillaryS; Sebia, Montpellier, France), using fresh hemolysate detected an abnormal variant and an elevated Hb F (Figure 1). Glucose-6-phosphate dehydrogenase (G6PD) screening and direct Coombs tests were negative. The parents’ blood smears, complete blood cell (CBC) tests and Hb electrophoresis, were all negative.