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Drug Allergy
Published in Pudupakkam K Vedanthan, Harold S Nelson, Shripad N Agashe, PA Mahesh, Rohit Katial, Textbook of Allergy for the Clinician, 2021
Type II hypersensitivity drug reactions are uncommon. Initially a drug binds to and acts as a hapten, stimulating IgG or IgM production to that foreign hapten-protein complex. Subsequent binding of the drug to preformed IgG or IgM causes cell destruction by macrophage- or complement-mediated lysis. These reactions usually occur with high-dose and prolonged treatment courses. Immune-induced thrombocytopenia may occur following treatment with heparin, quinidine, propylthiouracil, gold salts, beta-lactams sulfonamides, vancomycin and other drugs. Membrane damage occurs due to drug–antibody complexes, which are adsorbed onto cell membranes. Type II reactions are usually associated with a positive direct and indirect Coombs test.
Blood transfusion and rhesus disease
Published in Michael J. O’Dowd, The History of Medications for Women, 2020
The Rho (D) negative patient was known to be at risk and routine examination of her serum for anti-Rh antibodies was introduced based on a test perfected by Coombs, Mourant and Race (1945) of Cambridge University. They discovered a method for detecting the presence of weak and ‘incomplete’ Rh antibodies. The test, also known as the indirect globulin method, became the eponymous ‘Coombs test’. If antibodies were found in the maternal serum, a quantitative test was carried out to determine the titer of antibody. Many studies reported that the strength and changes in antibody Rh titers could be useful in determining how the pregnancy should be managed and whether preterm delivery could effect a satisfactory outcome for the fetus.
Waldenström Macroglobulinemia
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
IgM paraprotein directed to erythrocyte antigens can lead to autoimmune hemolytic anemia. While 10% of patients may have a positive Coombs test, only 3% will develop significant hemolysis [57]. Immune thrombocytopenic purpura and acquired von Willebrand disease are rare but well-described manifestations in WM [65]. Cryoglobulinemia, manifested by Raynaud phenomenon, acrocyanosis, peripheral neuropathy, vasculitis, or renal failure, is the result of IgM immune-complex precipitation [66]. Cold agglutinin disease, manifested by extravascular hemolytic anemia after cold exposure, is the result of IgM binding to RBC surface antigens [67]. Both manifestations have been described in approximately 10% of patients with WM [57].
A family case series of inherited thrombocytopenia
Published in Baylor University Medical Center Proceedings, 2023
Artur Borkowski, Jakub Gawryś, Gracjan Iwanek, Jarosław Dybko
On admission, thrombocytopenia was reported in every subject, ranging from 19 to 75 × 109/L (Table 2). Evaluation of platelet function by measuring occlusion time with epinephrine and collagen was not possible because the platelet count was too low. Blood smear examination revealed a normal size and morphology of platelets in every proband. The results of the coagulation tests are presented in Table 2. C-reactive protein, total bilirubin, indirect bilirubin, lactate dehydrogenase, and haptoglobin were normal in all probands. Direct and indirect Coombs test was evaluated as negative in each patient. Abdominal ultrasound examination in each proband revealed a spleen of average size 8.3 × 3.9 cm with homogeneous parenchyma. Bone marrow biopsy reported slightly reduced cellularity of bone marrow, erythroid normoblastic hypoplasia, normal myelopoiesis, and decreased megakaryocyte number with primarily immature forms in proband III2 and normocellular bone marrow with normal erythropoiesis, myelopoiesis, and megakaryocytes in individual III9.
Targeted next-generation sequencing revealed a novel homozygous mutation in the LRBA gene causes severe haemolysis associated with Inborn Errors of Immunity in an Indian family.
Published in Hematology, 2022
Prabhakar Kedar, Rashmi Dongerdiye, Shanmukhaiah Chandrakala, Umair Ahmed Bargir, Manisha Madkaikar
Written informed consent was taken for blood collection and DNA analysis from the patient's parents as a patient is a minor and for the publication of this report, as per the protocol of the institutional ethics review board of NIIH Mumbai.The clinical history of the patient was obtained from the previous medical records. She was referred to our Institute with major complaints of low haemoglobin (Hb-6.7 g/dl), yellowish discoloration of eyes and sclera. There was a history of jaundice on 3rd day of life, which was relieved with phototherapy. Parents also observed that child had weakness and used to be irritable intermittently. For 2 years, she had received ten units of packed red cells transfusions. Her peripheral blood smear revealed anisopoikilocytes and a few microspherocytes and a few nRBC. The patients’ Coombs test (DAT) was strongly positive. The reticulocyte count was 40%, and the indirect bilirubin level was 6.15 mg/dl (total bilirubin level was 7.14 mg/dl).
Two cases of methaemoglobinaemia and haemolysis due to poisoning after skin absorption of 4-chloro-1-nitrobenzene
Published in Clinical Toxicology, 2022
Guangcai Yu, Yaqian Li, Siqi Cui, Tianzi Jian, Baotian Kan, Xiangdong Jian
Methaemoglobinaemia is caused by the conversion of ferrous iron to the ferric state in red blood cells, resulting in tissue hypoxia and damage [4]. Herein, the responsible toxic substance was 4-chloro-1-nitrobenzene, and methaemoglobin levels at admission in patients 1 and 2 were 78.6% and 63.6%, respectively. The powdered debris of 4-chloro-1-nitrobenzene was mainly exposed to the patients' lower limb skin. We observed oxidative haemolysis 9 days after exposure; this was associated with elevated indirect bilirubin levels, decreased haemoglobin and reticulocyte counts, and the appearance of bite and fragmented cells [5]. After cautiously administering MB, patients’ methaemoglobin levels reduced significantly; therefore, G6PD deficiency was not involved in haemolysis [6]. The Coombs test also showed no possibility of immune haemolytic anaemia. Sulphhaemoglobin and methaemoglobin are abnormal products of haemoglobin, which can be produced by the same substances, resulting in haemolytic anaemia [7]. The arterial blood-gas analysis indicated that oxyhaemoglobin levels were not completely restored, despite methaemoglobin reduction to normal levels; this may have been due to the observed haemolysis. Hence, we speculate that the haemolysis was likely secondary to the toxic effects of 4-chloro-1-nitrobenzene-related methaemoglobin and sulphhaemoglobinaemia.