Unexplained Fever In Hematologic Disorders Section 1. Benign Hematologic Disorders
Benedict Isaac, Serge Kernbaum, Michael Burke in Unexplained Fever, 2019
A distinct form of severe anemia, usually normocytic or mildly macrocytic, is aplastic anemia,51 due to bone marrow failure. The peripheral blood usually shows anemia associated with leukopenia and thrombocytopenia (pancytopenia). The reticulocyte count is low. The bone marrow biopsy will reveal absent or reduced cellularity. The serum iron is elevated. The clinical manifestations vary with the severity of the pancytopenia. Bleeding and infection are the most common complications. Infection is usually associated with fever, but local signs of inflammation may be absent. The differential diagnosis is important because pancytopenia may occur in a variety of disorders, potentially evolving with fever, including lymphoma, pre-leukemia, metastatic carcinoma to the marrow, granulomatous diseases, parenchymal liver diseases with hypersplenism, and lipidoses. Two other diseases, usually febrile, SLE and tuberculosis due to atypical mycobacteria, may be associated with pan-yctopenia; some viral diseases, especially hepatitis and infectious mononucleosis, have also been implicated. The main causes of aplastic anemia are ionizing irradiation and chemical agents. The latter include chloramphenicol, phenylbutazone, hydantoin, sulfa drugs, phe-nothiazines, quinacrune, colchicine, sulfonylureas, gold compounds, benzene, insecticides, organic arsenicals, antineoplastic, and immunosuppressive drugs.
Diamond-Blackfan Anemia
Stephen A. Feig, Melvin H. Freedman in Clinical Disorders and Experimental Models of Erythropoietic Failure, 2019
If the diagnosis of DBA versus TEC is in doubt, transfusion should be modest, to a hemoglobin level of 7 to 8 g/dl, to avoid suppression of erythropoiesis, which might delay recovery in those patients who have TEC. In 1951 Gasser found that corticosteroid treatment with adrenocorticotropic hormone (ACTH) was effective, having noted erythroblastopenia in patients with transient allergic disorders, and observing increased eosinophils in the marrow of patients with DBA.91,144,145 Allen and Diamond reported remission in 12 of 22 patients.146 The initial drugs were cortisone and ACTH. We now recommend starting patients on prednisone, 2 mg/kg/day orally in three or four divided doses. A reticulocyte response usually occurs within 1 to 2 weeks; if there is no response, the dose can be increased to 3 to 6 mg/kg/day. Occasionally a different cortisteroid, such as dexamethasone, may be effective. Marrow-suppressant drugs or those that might affect the metabolism of prednisone, such as phenytoin or phenobarbital, should be discontinued. Allen and Diamond146 found that the response was better if the interval between diagnosis and treatment was short, but this may have been because hemosiderosis had developed during the interval if a sufficiently large number of transfusions were given. Steroid-induced remissions have been seen after 10 years of transfusions.50,55,147 A history of transfusions should thus not preclude an adequate trial of steroids coupled with iron chelation.
Haemato-Oncology
John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie in Basic Sciences Endocrine Surgery Rhinology, 2018
All basic laboratory haematology is fully automated. The full (or complete) blood count (FBC) will give the requesting clinician the following:57The number of red cells (red cell count, RCC), their size (mean corpuscular volume, MCV) and the haemoglobin concentration within them (mean corpuscular haemoglobin, MCH or mean corpuscular haemoglobin concentration, MCHC).58 It will also measure the haemoglobin concentration (Hb) within whole blood. The haemoglobin can be low and the red cell count high if those red cells don’t contain much haemoglobin within them (as in certain haemoglobinopathies). Most analysers will also offer a reticulocyte count. Reticulocytes are simply young red cells recently made in the marrow and if the reticulocyte count is high then the marrow is making lots of red cells.The total white cell count; and usually a 5-part differential giving the number of neutrophils, monocytes, lymphocytes, eosinophils and basophils. The counters discriminate these cells on the basis of size and staining.The platelet count.The analysers are good but abnormalities may be confirmed after examination of the blood smear. Samples that are clotted or diluted give erroneous results and should be repeated. Similarly samples that are not freshly analysed may give inaccurate results.
Transcranial Doppler Velocities among Sickle Cell Disease Patients in Steady State
Published in Hemoglobin, 2020
Khaled Salama, Rasha Rady, Rania H. Hashem, Mona El-Ghamrawy
Data were collected by interviewing the patients and through review of the medical records. Venous sampling was done for complete blood count (CBC); results were obtained with an automated counter (XS 800 I; Sysmex Corporation, Kobe, Japan), reticulocyte count was measured by staining reticulocytes by supra vital stain, a stain that has high affinity to ribosomal proteins. The preparation used was 1 mL of sample mixed with 1 ml of the stain (1:1) for 20 min., then a fresh film was prepared. The reticulocyte count was expressed in relation to 1000 counted erythrocytes (i.e. %). Serum ferritin was measured by enzyme-linked immunosorbent assay (ELISA) techniques using AxSYM™ (Abbott Laboratories, Abbott Park, IL, USA). Chemistry panel included serum bilirubin for total bilirubin, alanine transaminase (ALT), aspartate aminotransferase (AST) and serum lactate dehydrogenase (LDH) were measured using a Beckman Coulter counter (Miami, FL, USA). Serum was physically separated from contact with cells as soon as possible, with a maximum time limit of 2 hours from the time of collection.
Fist Detection of Hb Suqian[β42(CD1) Phe→Leu, HBB:c.129T > A] in Han Chinese
Published in Hemoglobin, 2023
Wenjuan Wang, Zixuan Ding, Airui Jiang, Suning Chen, Huanju Han
The proband was a female who was 62 years old. She came to our hospital because of chronic anemia and jaundice. The hematological parameters were obtained on a hematology analyzer(Sysmex Corporation, Kobe, Japan) as follows: red blood cell (RBC) count 3.55 × 1012/L (reference range 3.50–5.50 × 1012/L), Hb 10.0 g/dL (reference range 11.5–15.0 g/dL), mean corpuscular volume (MCV) 94.9 fL (reference range 82.0–100.0 fL), mean corpuscular Hb (MCH) 28.0 pg (reference range 27.0–33.5 pg), and reticulocyte count 9.5% (reference range 0.5%–1.5%). Bilirubin, haptoglobin(HP) and lactate dehydrogenase (LDH) were determined by an automatic analyzer (Hitachi Ltd., Tokyo, Japan) and the results revealed hemolysis occurred [HP < 3.0 mg/dL; total bilirubin (TBIL) 60.44 μmol/L; indirect bilirubin (IBIL) 45.14 μmol/L; LDH 480.8 U/L].
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
Patients with an unstable β chain variant usually develop symptoms at around 1 month after birth when adult Hb begins to be the predominant Hb because of the γ to β chain switch shortly after the time of birth [9]. Jaundice would be the most obvious clinical manifestation with or without a mild anemia. However, anemia can be exacerbated following an acute infection or exposure to oxidant medications. The early diagnosis of an unstable Hb variant is suspected in infants with jaundice caused by unexplained hemolytic anemia. In such patients with anemia the other cell lines are unaffected and the reticulocyte count is increased. Typical findings on the blood smear may include anisocytosis, prominent basophilic stippling, and/or microspherocytes. The presence of Heinz bodies is consistent with the diagnosis but not required [10]. As most unstable Hbs cannot be detected by HPLC or other means of Hb separation, a negative test in the setting of a consistent clinical picture does not eliminate the diagnosis [11]. DNA sequencing of the α- and β-globin genes should be pursued. It is the most definitive means of establishing the diagnosis and characterizing the variant.
Related Knowledge Centers
- Microscope
- Ribosomal Rna
- Bone Marrow
- Circulatory System
- Cell Nucleus
- Red Blood Cell
- Erythropoiesis
- Staining
- New Methylene Blue
- Romanowsky Stain