Cold antibody hemolytic anemia – known by the acronym Cad (Cold agglutinin disease) – is a “hemolytic anemia” in which there is a “reduction of red blood cells, and therefore of hemoglobin values, linked to their destruction by autoantibodies. It is defined by cold antibodies because these antibodies produced by the subject's body” against its own red blood cells “are activated in the temperature range between 3 and 4 degrees”. It is a “rare and underestimated pathology. A study from a few years ago”, in particular, “showed that in the colder regions, therefore in Northern Europe, there was a prevalence, therefore a difference in the onset of the pathology in the population general, 20 cases compared to 5 in Lombardy, therefore 20 cases in Northern Europe compared to 5 in Lombardy per million inhabitants”.
Thus Luana Fianchi, from the Diagnostic Imaging and Oncology Radiotherapy Department – Hematology and Hematopoietic Stem Cell Transplant Unit of the Agostino Gemelli Irccs University Hospital Foundation in Rome, speaking today in the capital with a video message at the presentation event of 'A life without winter' , the diary that tells the patients' wishes, born from an initiative by Sanofi in collaboration with FB&Associati, with the contribution of Cittadinanzattiva and Uniamo, the Italian Federation of rare diseases.
“In the temperature range between 3 and 4 degrees – explains the expert – the autoantibodies”, i.e. the antibodies that mistakenly attack the body's own cells, “are activated and become able to recognize some substances that are on the red blood cells of the patient and to agglutinate them”, that is, to bind them. A situation which, in turn, activates the “complement – which is very important for the immune response in general, against infections – which leads to a destruction of the red blood cells which can be more or less marked”.
The symptoms that the patient feels “are certainly extreme asthenia – describes Fianchi – intense fatigue even in carrying out normal daily activities, in addition to the signs of hemolysis, therefore the patient reports having, for example, very dark urine precisely because the destruction of the red blood cells then leads to the elimination of hemolysis substances. You can have jaundice, therefore a yellowish color of the skin, but certainly among the most disabling symptoms are fatigue and asthenia”. These can be associated with disabling conditions, for example, in the extremities. With exposure to the cold, many of these patients can have a so-called Raynaud's phenomenon: cyanosis of the hands or feet and, in the most serious cases, it can also lead to thrombotic and ischemic events of the extremities”.
Knowledge of this disease “is important because – underlines the haematologist – it also means giving extra guarantees to these patients. For Cad, for example, there are no patient associations that can turn to the authorities” to carry forward their requests , as an exemption code for the pathology, but also “to share experiences both in everyday life and in the workplace. I believe that the diary will help to share and raise awareness of this type of pathology which can be very disabling for the patient and can have also an important burden for the caregivers, the people who are close to these patients who often need continuous assistance for checks in hospital. The fact of being accompanied for therapy or checks – concludes Fianchi – is a very important data which then reflects on the entire society.”
#Rare #diseases #Hips #Gemini #Autoimmune #hemolytic #anemia #affects #North
