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PCH is a rare autoimmune hemolytic anemia (AIHA) but is one of the most common causes of AIAH in children. For the diagnosis, it is important to perform the appropriate methods of serological investigation and show the typical biphasic reaction. This is a case report of a child who presented with features of haemolysis and was diagnosed with PCH of...
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... 12 PCH in children now more commonly occurs as an acute transient haemolysis secondary to infections (>70% post respiratory infection), vaccinations, haematological malignancies and autoimmune disorders (figure 6). 1 4 12-14 Often the haemolysis in PCH is severe because it is intravascular; however, spontaneous resolution over several weeks, once the infection has subsided, is common. 15 The autoantibody associated with PCH is the D-L antibody after its discoverers in 1904. An indirect D-L test is the only specific diagnostic tool available for PCH. ...
Paroxysmal cold haemoglobinuria (PCH) accounts for around a third of cases of autoimmune haemolytic anaemia in children. PCH is caused by an autoantibody that fixes complement to red cells at low temperatures and dissociates at warmer temperatures (a biphasic haemolysin), triggering complement-mediated intravascular haemolysis. Named the Donath-Landsteiner (D-L) antibody after its discoverers, it is usually formed in response to infection and demonstrates specificity for the ubiquitous red cell P-antigen. A D-L test can be used to detect the presence of the D-L autoantibody in the patients’ serum. Here we discuss the use of the D-L test in identifying PCH in a 2-year-old boy who presented with haemolytic anaemia. A summary of the key information can be found in the infographic.
... Paroxysmal cold haemoglobinuria (PCH) is mediated by biphasic haemolysins, which sensitise red blood cells in a cold environment and cause direct intravascular haemolysis when the red blood cells reach the temperature of 37 o C [1,2]. Acute attacks of anaemia are often severe, but in most cases only supportive care is required [1][2][3][4][5]. ...
... Paroxysmal cold haemoglobinuria (PCH) is mediated by biphasic haemolysins, which sensitise red blood cells in a cold environment and cause direct intravascular haemolysis when the red blood cells reach the temperature of 37 o C [1,2]. Acute attacks of anaemia are often severe, but in most cases only supportive care is required [1][2][3][4][5]. Kidney injury due to PCH in children is rare [6][7][8][9]. ...
... PCH typically occurs in young children. The incidence ranges from 5.1% to 10%, and the boy-to-girl ratio is 2.1 : 1 [1][2][3][4]. Almost all cases are acute and transient. ...
Paroxysmal cold haemoglobinuria (PCH) is a form of autoimmune haemolytic anaemia (AIHA) characterised by a sudden onset of haemoglobinuria, either spontaneously or following exposure to cold. In children, it is commonly seen following a viral illness or after immunisation. Diagnosis of PCH is confirmed by a positive Donath Landsteiner (DL) test in which biphasic haemolysins are detected. However, in a real clinical setting, the serological diagnosis of PCH is not always easy. PCH can cause tubular renal injury, which in turn can lead to renal impairment. We describe a case of a two-year-old boy who was admitted to the hospital with pallor, jaundice, dehydration, and dark urine. Two weeks before admission, the child had an upper respiratory tract infection. Laboratory tests showed severe anaemia (haemoglobin 4.5g/dl, haematocrit 11.5%, LDH 8525 U/l), hyperbilirubinaemia (104 μmol/l), haemoglobinuria, and acute kidney injury: GFR 43.9 ml/min/1.73 m² (grade 2 according to Acute Kidney Injury Network). The direct antiglobulin test was positive for C3c and C3d complement components. The diagnosis of PCH was confirmed by the presence of biphasic antibodies in a DL test on the third day of hospitalisation. The patient received supportive treatment.
In addition to the patient’s clinical history, an unusual characteristic may provide a clue as to the nature of an antibody. Solve it!
Paroxysmal cold hemoglobinuria (PCH) is a very rare subtype of autoimmune hemolytic anemia caused by the presence of cold-reacting autoantibodies in the blood and characterized by the sudden presence of hemoglobinuria, typically after exposure to cold temperatures. The acute onset PCH occurs following viral illnesses whilst the chronic form is secondary to hematological malignancies and tertiary syphilis. It is a complement mediated intravascular hemolytic anemia associated with a biphasic antibody against the p antigen on red cells. We describe a three-year child who had acute onset PCH following likely viral infection. The diagnosis was confirmed by demonstration of strongly positive Donnath Landsteiner antibodies. She made a gradual recovery with supportive treatment, ten days following the initial detection of hemolysis. Parents were educated about the need to avoid cold exposure to prevent precipitation of further hemolysis and folic acid was commenced to assist the recovery of erythropoiesis.International Journal of Human and Health Sciences Vol. 06 No. 01 January’22 Page: 147-149
Paroxysmal cold hemoglobinuria (PCH) is a very rare subtype of autoimmune hemolytic anemia caused by the presence of cold-reacting autoantibodies in the blood and characterized by the sudden presence of hemoglobinuria, typically after exposure to cold temperatures. The acute onset PCH occurs following viral illnesses whilst the chronic form is secondary to hematological malignancies and tertiary syphilis. It is a complement mediated intravascular hemolytic anemia associated with a biphasic antibody against the P antigen on red cells. We describe a three year child who had acute onset PCH following likely viral infection. The diagnosis was confirmed by demonstration of strongly positive Donnath Landsteiner antibodies. She made a gradual recovery with supportive treatment, ten days following the initial detection of haemolysis. Parents were educated about the need to avoid cold exposure to prevent precipitation of further haemolysis and folic acid was commenced to assist the recovery of erythropoiesis.
A 5-year-old boy presents to the emergency department (ED) with 2 days of fever (temperature 100.7 °F), fatigue, jaundice, and dark urine. The parents note that the child had symptoms of a viral upper respiratory illness 2 weeks before presentation and that he was previously healthy with no history of blood transfusion. In the ED, the child weighs 21.3 kg, and laboratory workup reveals severe anemia with hemoglobin (Hgb) 5.4 g/dL, elevated total bilirubin 5.1 mg/dL, aspartate aminotransferase (AST) 357 U/L (normal range, 8–60 U/L), and lactate dehydrogenase (LDH) 8126 U/L. Serum haptoglobin is undetectable. Urinalysis results are as follows: color, dark; red blood cells (RBCs), 0–3 per high-power field; and urine Hgb, 3+ positive. Notably, the onset of symptoms (fever, fatigue, jaundice, dark urine) was associated with eating a bowl of ice cream. An ethylenediaminetetraacetic acid (EDTA) anticoagulant type and screen sample is submitted to the blood bank along with a request for RBC transfusion.
