Lymphoproliferative Disorders of The Lung
Lourdes R. Laraya-Cuasay, Walter T. Hughes in Interstitial Lung Diseases in Children, 2019
AILD usually affects elderly patients with average onset of illness at 60 years of age. The onset may be acute or insidious. However, there are very few reports of onset in childhood.44-47 The clinical description of the disease in children is similar to that in the older age group. Constitutional symptoms, hepatosplenomegaly, skin rashes, anemia with positive Coombs’ test and polyclonal hypergammaglobulinemia are present. In the four children described in literature and in one boy whom the author has cared for over 5 years,48 the clinical findings were generalized lymphadenopathy, hepatosplenomegaly, fever, pruritus, skin rash which was malar usually and intermittent, hyperhidrosis especially at night, and progressive weight loss. All the children were male. The earliest onset was at 2V2 months in the reported cases. The onset in our patient was at 8 months. Polyclonal hypergammaglobulinemia, anemia and leukocytosis were found in all children. A positive Coombs’ test was found in one half of all reported cases. A normochromic normocytic anemia was present in 84% of patients and only one third of these patients had positive Coombs’ test. Diagnostic lesions were found in sections of bone marrow core biopsies of spicules closely resembling the lymph node histologically.42
Benzylpenicillin (Penicillin G)
M. Lindsay Grayson, Sara E. Cosgrove, Suzanne M. Crowe, M. Lindsay Grayson, William Hope, James S. McCarthy, John Mills, Johan W. Mouton, David L. Paterson in Kucers’ The Use of Antibiotics, 2017
The uncommon complication of hemolytic anemia may occur when i.v. Pen G in a dose usually greater than 6 g daily is given to patients who have previously received large doses of the drug (White et al., 1968). Pen G–induced hemolytic anemia is of the hapten type, i.e. the antibody produced is directed to the drug (hapten). A strongly positive direct antiglobulin reaction (Coombs test) is the main diagnostic feature, which is due to induced IgG antibody reacting with Pen G–coated red cells (Garratty and Petz, 1975; Axelson and Lobuglio, 1980). Erythrocytes not coated with Pen G may also be destroyed because they may bind activated complement components and thereby be susceptible to premature destruction by the reticuloendothelial system (Kerr et al., 1972).
Drug Allergy
Pudupakkam K Vedanthan, Harold S Nelson, Shripad N Agashe, PA Mahesh, Rohit Katial in 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.
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).
Coombs-positive refractory acquired thrombotic thrombocytopenic purpura in a patient with chronic myelomonocytic leukemia successfully treated with rituximab
Published in Acta Clinica Belgica, 2020
Ivan Krecak, Marijana Grgic Medic, Velka Gveric–Krecak, Pavle Roncevic, Sandra Bašić Kinda, Jaksa Babel, Radovan Radonic
aTTP associated with a positive direct Coombs test is also a rare entity. Very few cases of aTTP with a positive Coombs test have been reported, and most of them were associated with SLE [17]. A false‐positive direct Coombs test is also possible with drugs such as penicillin, cephalosporins, methyldopa and after blood transfusion, but our patient had received none of them. ES is also an uncommon disorder caused by auto-antibodies directed against self erythrocytes and platelets. The pathophysiology of the disease involves phagocytosis of auto-antibody-coated erythrocytes and platelets in the spleen and complement-mediated hemolysis. Positive direct Coombs test is considered to be the hallmark of the disease. Mild splenomegaly is a frequent finding. ES can be idiopathic or secondary, due to lymphoproliferative, rheumatic and chronic inflammatory disorders (i.e. SLE) [18]. Workup for all these disorders in our case returned negative.
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.