Kuliszkiewicz-Janus Malgorzata, Bednarek-Tupikowska Grazyna, Rózycka Beata, Dereń Izabela
Katedra i Klinika Hematologii, Nowotworów Krwi i Transplantacji Szpiku AM, Wrocławiu.
Pol Arch Med Wewn. 2004 Nov;112(5):1311-9.
Addison-Biermer's anaemia is an autoimmune disease. It may coexist with other auto-aggressive diseases, precede them or join the other existing autoimmune diseases. It most often accompanies the Hashimoto disease but also may coexist polyglandular autoimmune syndrome (PGA). Three types of PGA are distinguished: PGA1--Blizzard's Syndrome, PGA2--Schmidt's Syndrome, and PGA3. The latter, unlike the remaining ones, is characterized by normal function of adrenal glands. Addison-Biermer's anaemia occurrence may be often difficult to diagnose as coexisting illnesses might ouflage its clinical symptoms. The aim of this paper was to analyse patients with different types of PGA with coexisting Addison-Biermer's anaemia. Group of 24 individuals was analysed: 2 women with PGA1, 10 patients with PGA2, 10 patients with PGA3. In 2 remaining ones PGA was not confirmed. Addison-Biermer's anaemia occurred in 7 patients (2 with PGA2 and 5 with PGA3 syndrome). Decreased concentration of vitamin B12 was diagnosed in 3 individuals among 24 examined patients (1 with type 3 and 2 with type 2), as well in 2 patients with unconfirmed PGA. Addison-Biermer's anaemia was not observed in patients with PGA1. We observed that megaloblastic anaemia occurred characteristic schedule depending on appearance of autoimmune diseases: in PGA2--many years after other immunopathies were found, in PGA3--as first auto-aggressive disease. Our analysis suggests the necessity of detailed check-ups on patients with Addison-Biermer's anaemia, as with time they may develop other diseases, especially hypothyroidism and/or PGA failure. On the contrary, in individuals with thyroid gland diseases and PGA syndromes further checkups should be megaloblastic anaemia-sensitive. In both cases it is important to consider substitutive treatment. The possibility of family coexisting both pernicious anaemia and autoimmune endocrinopathies needs diagnostics of members of the patient's family.
艾迪生 - 比尔默贫血是一种自身免疫性疾病。它可能与其他自身攻击性疾病共存、先于它们出现或加入现有的其他自身免疫性疾病。它最常伴随桥本氏病,但也可能与多腺体自身免疫综合征(PGA)共存。PGA分为三种类型:PGA1——暴雪综合征,PGA2——施密特综合征,以及PGA3。与其他类型不同,后者的特征是肾上腺功能正常。艾迪生 - 比尔默贫血的发生可能常常难以诊断,因为共存的疾病可能掩盖其临床症状。本文的目的是分析患有不同类型PGA且伴有艾迪生 - 比尔默贫血的患者。分析了24名个体:2名患有PGA1的女性,10名患有PGA2的患者,10名患有PGA3的患者。其余2名患者未确诊为PGA。7名患者出现了艾迪生 - 比尔默贫血(2名患有PGA2,5名患有PGA3综合征)。在24名接受检查的患者中有3名个体被诊断出维生素B12浓度降低(1名患有3型,2名患有2型),另外2名未确诊PGA的患者也出现了这种情况。患有PGA1的患者未观察到艾迪生 - 比尔默贫血。我们观察到巨幼细胞贫血根据自身免疫性疾病的出现呈现出特征性的发病时间:在PGA2中——在发现其他免疫病多年后出现,在PGA3中——作为首发的自身攻击性疾病。我们的分析表明,对患有艾迪生 - 比尔默贫血的患者进行详细检查是必要的,因为随着时间推移他们可能会患上其他疾病,尤其是甲状腺功能减退和/或PGA功能衰竭。相反,对于患有甲状腺疾病和PGA综合征的个体,进一步的检查应该对巨幼细胞贫血敏感。在这两种情况下,考虑替代治疗都很重要。患者家族中同时存在恶性贫血和自身免疫性内分泌病的可能性需要对患者家族成员进行诊断。