Secondary cold agglutinin syndrome: Difference between revisions

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::*[[Mixed autoimmune hemolytic anemia|Mixed cold- and warm-antibody type]]  
::*[[Mixed autoimmune hemolytic anemia|Mixed cold- and warm-antibody type]]  


<ref name="Berentsen Sundic 2015 pp. 1–11">{{cite journal | last=Berentsen | first=Sigbjørn | last2=Sundic | first2=Tatjana | title=Red Blood Cell Destruction in Autoimmune Hemolytic Anemia: Role of Complement and Potential New Targets for Therapy | journal=BioMed Research International | publisher=Hindawi Limited | volume=2015 | date=2015-01-29 | issn=2314-6133 | pmid=25705656 | pmc=4326213 | doi=10.1155/2015/363278 | pages=363278}}</ref>
<ref name="Berentsen Sundic 2015 pp. 1–11">{{cite journal | last=Berentsen | first=Sigbjørn | last2=Sundic | first2=Tatjana | title=Red Blood Cell Destruction in Autoimmune Hemolytic Anemia: Role of Complement and Potential New Targets for Therapy | journal=BioMed Research International | publisher=Hindawi Limited | volume=2015 | date=2015-01-29 | issn=2314-6133 | pmid=25705656 | pmc=4326213 | doi=10.1155/2015/363278 | pages=1–11}}</ref>


==Signs and symptoms==
==Signs and symptoms==

Revision as of 03:27, 14 March 2019

Secondary cold agglutinin syndrome (Secondary CAS, CAS) is far more uncommon than primary cold agglutinin disease (Primary CAD, CAD). Among 295 consecutive individuals with autoimmune hemolytic anemia (AIHA) described retrospectively by Dacie in a single-center series, 7 patients (2.4%) were classified as having CAS secondary to malignant disease. [1]

Classification

Autoimmune hemolytic anemia.

[3]

Signs and symptoms

In occasional patients, high-titer, high-thermal amplitude productions of cold antibodies (CA) result in hemolytic anemia which is transient but can be severe. [4][5]

Etiology

CAS has been described in patients diagnosed with diffuse large B-cell lymphoma, Hodgkin's lymphoma, carcinomas, sarcomas, metastatic melanoma, and chronic myeloproliferative disorders. [6] Some of these associations have been poorly documented[4], and the most convincing association with malignant disease has been described with non-Hodgkin's lymphoma. [7][8][9][10]

Pathophysiology

In CAS complicating aggressive lymphoma, the pathological cold sensitive antibodies are monoclonal, most often IgM, and have anti-I specificity. In contrast to pathological cold sensitive antibodies found in primary CAD, however, the Immunoglobulin light chain restriction can be λ as well as κ. [7][8]

Polyclonal anti-I specific pathological cold sensitive antibodies of the IgM class are produced as part of the physiological immune response in Mycoplasma pneumoniae pneumonia. They do not usually give rise to significant hemolysis. In occasional patients, however, production of high-titer, high-thermal amplitude, pathological cold sensitive antibodies results in hemolytic anemia which is transient but can be severe. [4][5][11]

CAS complicating mycoplasma pneumoniae infection has been reported to account for approximately 8% of AIHA. [1] Still more uncommon but less severe, polyclonal anti-i specific CA of the IgM or IgG class can result in CAS in Epstein-Barr virus infection [4][12]. Transient CAS has also been described following cytomegalovirus infection, varicella, rubella, adenovirus infection, influenza A, Legionella pneumophila pneumonia, listeriosis, and pneumonia caused by Chlamydia species. [4]

Figure 1

In CAS secondary to infection or aggressive lymphoma, the Red blood cell breakdown is complement-dependent, mediated by exactly the same mechanisms as in primary cold agglutinin disease (See Figure 1). [4][13]

Management

Treatment of the underlying disease, if relevant and available, is often the only possible drug therapy for the hemolytic complication. Corticosteroid therapy has been used but is not evidence-based. In severely anemic patients, blood transfusions can safely be given provided the same precautions are carefully observed as in primary cold agglutinin disease. [4]

Epidemiology

Among 295 consecutive individuals with AIHA described retrospectively by Dacie in a single-center series, 7 patients (2.4%) were classified as having CAS secondary to malignant disease. [1] CAS complicating mycoplasma pneumoniae infection has been reported to account for approximately 8% of AIHA. [1]

Reference

  1. ^ a b c d Dacie J. The auto-immune haemolytic anaemias: introduction. In: Dacie J., editor. The Haemolytic Anaemias. Vol. 3. London, UK: Churchill Livingstone; 1992. pp. 1–5.
  2. ^ Berentsen, Sigbjørn; Beiske, Klaus; Tjønnfjord, Geir E. (2007-07-21). "Primary chronic cold agglutinin disease: An update on pathogenesis, clinical features and therapy". Hematology (Amsterdam, Netherlands). 12 (5). Informa UK Limited: 361–370. doi:10.1080/10245330701445392. ISSN 1607-8454. PMC 2409172. PMID 17891600.
  3. ^ Berentsen, Sigbjørn; Sundic, Tatjana (2015-01-29). "Red Blood Cell Destruction in Autoimmune Hemolytic Anemia: Role of Complement and Potential New Targets for Therapy". BioMed Research International. 2015. Hindawi Limited: 1–11. doi:10.1155/2015/363278. ISSN 2314-6133. PMC 4326213. PMID 25705656.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  4. ^ a b c d e f g Berentsen, Sigbjørn; Tjønnfjord, Geir E. (2012). "Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia". Blood Reviews. 26 (3). Elsevier BV: 107–115. doi:10.1016/j.blre.2012.01.002. ISSN 0268-960X. PMID 22330255.
  5. ^ a b Linz, DH; Tolle, SW; Elliot, DL (1984). "Mycoplasma pneumoniae pneumonia. Experience at a referral center". The Western Journal of Medicine. 140 (6): 895–900. ISSN 0093-0415. PMC 1011124. PMID 6741120.
  6. ^ Sokol, RJ; Hewitt, S; Stamps, BK (1981-06-20). "Autoimmune haemolysis: an 18-year study of 865 cases referred to a regional transfusion centre". British Medical Journal (Clinical Research Ed.). 282 (6281): 2023–7. ISSN 0267-0623. PMC 1505955. PMID 6788179.
  7. ^ a b Crisp, D; Pruzanski, W (1982). "B-cell neoplasms with homogeneous cold-reacting antibodies (cold agglutinins)". The American Journal of Medicine. 72 (6): 915–22. doi:10.1016/0002-9343(82)90852-X. ISSN 0002-9343. PMID 6807086.
  8. ^ a b Dacie J. Haemolytic anaemias associated with malignant lymphomas other than Hodgkin's disease and chronic lymphocytic leukaemia (CLL) In: Dacie J., editor. The Haemolytic Anaemias.Vol. 4. London, UK: Churchill Livingstone; 1995. pp. 27–40.
  9. ^ NíÁinle, F.; Hamnvik, O.-P.R.; Gulmann, C.; Bermingham, C.; Kelly, J.; Mc Evoy, P.; Murphy, P. (2008). "Diffuse large B-cell lymphoma with isolated bone marrow involvement presenting with secondary cold agglutinin disease". International Journal of Laboratory Hematology. 30 (5). Wiley: 444–445. doi:10.1111/j.1751-553x.2007.00977.x. ISSN 1751-5521. PMID 18205841.
  10. ^ Eskazan, Ahmet Emre; Akmurad, Hamida; Ongoren, Seniz; Ozer, Ozden; Ferhanoglu, Burhan (2011). "Primary Gastrointestinal Diffuse Large B Cell Lymphoma Presenting with Cold Agglutinin Disease". Case Reports in Gastroenterology. 5 (2). S. Karger AG: 262–266. doi:10.1159/000328445. ISSN 1662-0631. PMC 3153337. PMID 21887126.
  11. ^ "Acute hemolytic anemia in primary atypical pneumonia associated with high titer of cold agglutinins; report of a case". The New England Journal of Medicine. 234 (25): 826–9. 1946-06-20. doi:10.1056/NEJM194606202342503. ISSN 0028-4793. PMID 20990099. {{cite journal}}: |first= missing |last= (help)
  12. ^ Wentworth, P; Bate, LR (1980-09-20), "Acute hemolytic anemia secondary to infectious mononucleosis.", Canadian Medical Association journal, 123 (6): 482–6, ISSN 0008-4409, PMC 1704830, PMID 7002274
  13. ^ Dacie J. Auto-immune haemolytic anaemia (AIHA): pathogenesis. In: Dacie J., editor. The Haemolytic Anaemias. Vol. 3. London, UK: Churchill Livingstone; 1992. pp. 392–451
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