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X-linked agammaglobulinemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Synonyms and keywords: Bruton agammaglobulinemia

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

X-linked agammaglobulinemia (also called X-linked hypogammaglobulinemia, XLA, Bruton type agammaglobulinemia) is a rare X-linked genetic disorder that affects the body’s ability to fight infection (origin of the name: A=no, gammaglobulin=Antibody). XLA patients do not generate mature B cells. B cells are part of the immune system and normally manufacture antibodies (also called immunoglobulins) which defends the body from infections (the humoral response). Patients with untreated XLA are prone to develop serious and even fatal infections.[1] Patients typically present in early childhood with recurrent infections, particularly with extracellular, encapsulated bacteria.[2] XLA is an X-linked disorder, and therefore is almost always limited to males. It occurs in a frequency of about 1 in 100,000 male newborns, and has no ethnic predisposition. XLA is treated by infusion of human antibody. Treatment with pooled gamma globulin cannot restore a functional population of B cells, but it is sufficient to reduce the severity and number of infections due to the passive immunity granted by the exogenous antibodies.[2]

XLA is caused by a mutation on the X chromosome of a single gene identified in 1993 and known as Bruton’s tyrosine kinase, or Btk.[2] XLA was first characterized by Dr. Ogden Bruton in a ground-breaking research paper published in 1952 describing a boy unable to develop immunities to common childhood diseases and infections. Bruton’s paper describes the first known immune deficiency. XLA is classified with other inherited (genetic) defects of the immune system, known as primary immunodeficiency disorders.[3]

References

  1. ↑ XLA information by St. Jude Children’s Hospital
  2. ↑ 2.0 2.1 2.2 X-Linked Agammaglobulinemia Patient and Family Handbook for The Primary Immune Diseases. Third Edition. 2001. Published by the Immune Deficiency Foundation
  3. ↑ Bruton, Ogden C. Agammaglobulinemia

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Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Historical Perspective

XLA was first characterized by Dr. Ogden Bruton in a ground-breaking research paper published in 1952 describing a boy unable to develop immunities to common childhood diseases and infections. Bruton’s paper describes the first known immune deficiency. XLA is classified with other inherited (genetic) defects of the immune system, known as primary immunodeficiency disorders.[1]

References

  1. ↑ Bruton, Ogden C. Agammaglobulinemia

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Classification

Classification

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References

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

XLA patients are specifically susceptible to viruses of the Enterovirus family, and mostly to: polio virus, coxsackie virus (hand, foot, and mouth disease) and Echoviruses. These may cause severe central nervous system conditions as chronic encephalitis, meningitis and death. An experimental anti-viral agent, pleconaril, is active against picornaviruses. XLA patients, however, are apparently immune to the Epstein-Barr virus (EBV), as they lack B cells needed for the viral infection.

It is not known if XLA patients are able to generate an allergic reaction, as they lack functional IgE antibodies.

Genetics

The gene Bruton’s tyrosine kinase (Btk) plays an essential role in the maturation B cells in the bone marrow, and when mutated, immature pre-B lymphocytes are unable to develop into mature B cells that leave the bone marrow into the blood stream. The disorder is X-linked (it is on the X chromosome), and is almost entirely limited to the sons of asymptomatic female carriers .[1] This is because males have only one copy of the X chromosome, while females have two copies; one normal copy of an X chromosome can compensate in for mutations in the other X chromosome. Females carriers have a 50% chance of giving birth to a male child with XLA.

An XLA patient will pass on the gene, and all of his daughters will be XLA carriers, meaning that any male grandchildren from an XLA patient’s daughters have a 50% chance of inheriting XLA. A female XLA patient can only arise as the child of an XLA patient and a carrier mother. XLA can also rarely result from a spontaneous mutation in the fetus of a non-carrier mother.



References

  1. ↑ X-Linked Agammaglobulinemia Patient and Family Handbook for The Primary Immune Diseases. Third Edition. 2001. Published by the Immune Deficiency Foundation

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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Causes

XLA is caused by a mutation on the X chromosome of a single gene identified in 1993 and known as Bruton’s tyrosine kinase, or Btk..[1] XLA was first characterized by Dr. Ogden Bruton in a ground-breaking research paper published in 1952 describing a boy unable to develop immunities to common childhood diseases and infections. Bruton’s paper describes the first known immune deficiency. XLA is classified with other inherited (genetic) defects of the immune system, known as primary immunodeficiency disorders.[2]

References

  1. ↑ X-Linked Agammaglobulinemia Patient and Family Handbook for The Primary Immune Diseases. Third Edition. 2001. Published by the Immune Deficiency Foundation
  2. ↑ Bruton, Ogden C. Agammaglobulinemia

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Differentiating X-linked agammaglobulinemia from other Diseases

Differentiating X-linked agammaglobulinemia from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Bruton’s agammaglobulinemia should be differentiated from other disorders leading to hypogammaglobulinemia and defects of humoral immunity.

Differentiating IgG deficiency from other Diseases

Bruton’s agammaglobulinemia should be differentiated from other disorders leading to hypogammaglobulinemia and defects of humoral immunity. The following conditions may be considered as differentials:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][24][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47][48][49][50]

Disorder Defect (Mechanism of Development) Characteristic Features Clinical Presentation Laboratory Findings
X-Linked (Bruton) Agammaglobulinemia
Selective IgA Deficiency
  • Serum IgA < 7 mg/dl
  • Normal IgG and IgM levels
Common Variable Immunodeficiency
  • Defective B cell differentiation
  • May be acquired in 20-30 years of age
Autosomal dominant hype IgE syndrome (Job’s Syndrome)
  • Distinctive coarse facies
  • Cold (non-inflammatory) Staphylococcal abscesses
  • Retained primary teeth
  • Eczema
Severe combined immunodeficiency (SCID)
Ataxia Telangiectasia
Hyper IgM Syndrome
Wiskott-Aldrich Syndrome
  • Malignancy: can cause the reduction in the immunoglobulin production.[51]
  • Viral infections: such as Epstein-Barr virus, HIV, cytomegalovirus are other causes of hypogammaglobulinemia..
  • Side effect of certain medications: Some drugs include systemic glucocorticoids, phenytoin, and carbamazepine, have been associated with IgG deficiency.[52]
  • Other causes of primary humoral immunodeficiencies.
  • Smoking: may cause IgG2 subclass deficiency.[53]
  • Protein-losing conditions: enteropathies, nephrotic syndrome, burns, and other traumas may cause abnormal loss of immunoglobulins.

References

  1. ↑ Agarwal S, Cunningham-Rundles C (September 2007). “Assessment and clinical interpretation of reduced IgG values”. Ann. Allergy Asthma Immunol. 99 (3): 281–3. doi:10.1016/S1081-1206(10)60665-5. PMC 3099256. PMID 17910333.
  2. ↑ Korthäuer U, Graf D, Mages HW, Brière F, Padayachee M, Malcolm S, Ugazio AG, Notarangelo LD, Levinsky RJ, Kroczek RA (February 1993). “Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM”. Nature. 361 (6412): 539–41. doi:10.1038/361539a0. PMID 7679206.
  3. ↑ Levy J, Espanol-Boren T, Thomas C, Fischer A, Tovo P, Bordigoni P, Resnick I, Fasth A, Baer M, Gomez L, Sanders EA, Tabone MD, Plantaz D, Etzioni A, Monafo V, Abinun M, Hammarstrom L, Abrahamsen T, Jones A, Finn A, Klemola T, DeVries E, Sanal O, Peitsch MC, Notarangelo LD (July 1997). “Clinical spectrum of X-linked hyper-IgM syndrome”. J. Pediatr. 131 (1 Pt 1): 47–54. PMID 9255191.
  4. ↑ Winkelstein JA, Marino MC, Ochs H, Fuleihan R, Scholl PR, Geha R, Stiehm ER, Conley ME (November 2003). “The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients”. Medicine (Baltimore). 82 (6): 373–84. doi:10.1097/01.md.0000100046.06009.b0. PMID 14663287.
  5. ↑ Subauste CS, Wessendarp M, Sorensen RU, Leiva LE (June 1999). “CD40-CD40 ligand interaction is central to cell-mediated immunity against Toxoplasma gondii: patients with hyper IgM syndrome have a defective type 1 immune response that can be restored by soluble CD40 ligand trimer”. J. Immunol. 162 (11): 6690–700. PMID 10352287.
  6. ↑ Hayward AR, Levy J, Facchetti F, Notarangelo L, Ochs HD, Etzioni A, Bonnefoy JY, Cosyns M, Weinberg A (January 1997). “Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM”. J. Immunol. 158 (2): 977–83. PMID 8993019.
  7. ↑ Davies EG, Thrasher AJ (April 2010). “Update on the hyper immunoglobulin M syndromes”. Br. J. Haematol. 149 (2): 167–80. doi:10.1111/j.1365-2141.2010.08077.x. PMC 2855828. PMID 20180797.
  8. ↑ Yel L (January 2010). “Selective IgA deficiency”. J. Clin. Immunol. 30 (1): 10–6. doi:10.1007/s10875-009-9357-x. PMC 2821513. PMID 20101521.
  9. ↑ Suzuki H, Kaneko H, Fukao T, Jin R, Kawamoto N, Asano T, Matsui E, Kasahara K, Kondo N (March 2009). “Various expression patterns of alpha1 and alpha2 genes in IgA deficiency”. Allergol Int. 58 (1): 111–7. doi:10.2332/allergolint.O-08-549. PMID 19153537.
  10. ↑ Cunningham-Rundles C (September 2001). “Physiology of IgA and IgA deficiency”. J. Clin. Immunol. 21 (5): 303–9. PMID 11720003.
  11. ↑ Edwards E, Razvi S, Cunningham-Rundles C (April 2004). “IgA deficiency: clinical correlates and responses to pneumococcal vaccine”. Clin. Immunol. 111 (1): 93–7. doi:10.1016/j.clim.2003.12.005. PMID 15093556.
  12. ↑ Chipps BE, Talamo RC, Winkelstein JA (April 1978). “IgA deficiency, recurrent pneumonias, and bronchiectasis”. Chest. 73 (4): 519–26. PMID 305332.
  13. ↑ Zinneman HH, Kaplan AP (September 1972). “The association of giardiasis with reduced intestinal secretory immunoglobulin A”. Am J Dig Dis. 17 (9): 793–7. PMID 5056860.
  14. ↑ Aghamohammadi A, Cheraghi T, Gharagozlou M, Movahedi M, Rezaei N, Yeganeh M, Parvaneh N, Abolhassani H, Pourpak Z, Moin M (January 2009). “IgA deficiency: correlation between clinical and immunological phenotypes”. J. Clin. Immunol. 29 (1): 130–6. doi:10.1007/s10875-008-9229-9. PMID 18683032.
  15. ↑ Janzi M, Kull I, Sjöberg R, Wan J, Melén E, Bayat N, Ostblom E, Pan-Hammarström Q, Nilsson P, Hammarström L (October 2009). “Selective IgA deficiency in early life: association to infections and allergic diseases during childhood”. Clin. Immunol. 133 (1): 78–85. doi:10.1016/j.clim.2009.05.014. PMID 19541543.
  16. ↑ Jacob CM, Pastorino AC, Fahl K, Carneiro-Sampaio M, Monteiro RC (May 2008). “Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper”. J. Clin. Immunol. 28 Suppl 1: S56–61. doi:10.1007/s10875-007-9163-2. PMID 18202833.
  17. ↑ Conley ME, Notarangelo LD, Etzioni A (December 1999). “Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies)”. Clin. Immunol. 93 (3): 190–7. doi:10.1006/clim.1999.4799. PMID 10600329.
  18. ↑ Mayer RJ, Schiffer CA, Peterson BA, Silver RT, Cornwell GG, McIntyre OR, Rai KR, Budman DR, Ellison RR, Maguire M (June 1985). “Intensive postremission therapy in adults with acute nonlymphocytic leukemia with ara-C by continuous infusion or bolus administration: preliminary results of a CALGB phase I study”. Semin. Oncol. 12 (2 Suppl 3): 84–90. PMID 4012343.
  19. ↑ Massaad MJ, Ramesh N, Geha RS (May 2013). “Wiskott-Aldrich syndrome: a comprehensive review”. Ann. N. Y. Acad. Sci. 1285: 26–43. doi:10.1111/nyas.12049. PMID 23527602.
  20. ↑ Candotti F (January 2018). “Clinical Manifestations and Pathophysiological Mechanisms of the Wiskott-Aldrich Syndrome”. J. Clin. Immunol. 38 (1): 13–27. doi:10.1007/s10875-017-0453-z. PMID 29086100.
  21. ↑ Sereni L, Castiello MC, Villa A (March 2018). “Platelets in Wiskott-Aldrich syndrome: Victims or executioners?”. J. Leukoc. Biol. 103 (3): 577–590. doi:10.1189/jlb.5MR0617-257R. PMID 28851742.
  22. ↑ Blundell MP, Worth A, Bouma G, Thrasher AJ (2010). “The Wiskott-Aldrich syndrome: The actin cytoskeleton and immune cell function”. Dis. Markers. 29 (3–4): 157–75. doi:10.3233/DMA-2010-0735. PMC 3835520. PMID 21178275.
  23. ↑ Bosticardo M, Marangoni F, Aiuti A, Villa A, Grazia Roncarolo M (June 2009). “Recent advances in understanding the pathophysiology of Wiskott-Aldrich syndrome”. Blood. 113 (25): 6288–95. doi:10.1182/blood-2008-12-115253. PMID 19351959.
  24. ↑ 24.0 24.1 Fischer A (November 2000). “Severe combined immunodeficiencies (SCID)”. Clin. Exp. Immunol. 122 (2): 143–9. PMC 1905779. PMID 11091267.
  25. ↑ Noguchi M, Yi H, Rosenblatt HM, Filipovich AH, Adelstein S, Modi WS, McBride OW, Leonard WJ (April 1993). “Interleukin-2 receptor gamma chain mutation results in X-linked severe combined immunodeficiency in humans”. Cell. 73 (1): 147–57. PMID 8462096.
  26. ↑ Puck JM (November 1996). “IL2RGbase: a database of gamma c-chain defects causing human X-SCID”. Immunol. Today. 17 (11): 507–11. PMID 8961626.
  27. ↑ Rowiński J, Souchier C, Czyba JC (1978). “DNA content of cells in human buccal smears. A preliminary study”. Acta Histochem. 62 (2): 276–81. doi:10.1016/S0065-1281(78)80093-2. PMID 104530.
  28. ↑ Morgan G, Levinsky RJ, Hugh-Jones K, Fairbanks LD, Morris GS, Simmonds HA (December 1987). “Heterogeneity of biochemical, clinical and immunological parameters in severe combined immunodeficiency due to adenosine deaminase deficiency”. Clin. Exp. Immunol. 70 (3): 491–9. PMC 1542189. PMID 3436096.
  29. ↑ Ballard RW, Cummings CW (August 1980). “Job’s syndrome”. Laryngoscope. 90 (8 Pt 1): 1367–70. PMID 7401839.
  30. ↑ Freeman AF, Holland SM (May 2008). “The hyper-IgE syndromes”. Immunol Allergy Clin North Am. 28 (2): 277–91, viii. doi:10.1016/j.iac.2008.01.005. PMC 2683262. PMID 18424333.
  31. ↑ Holland SM, DeLeo FR, Elloumi HZ, Hsu AP, Uzel G, Brodsky N, Freeman AF, Demidowich A, Davis J, Turner ML, Anderson VL, Darnell DN, Welch PA, Kuhns DB, Frucht DM, Malech HL, Gallin JI, Kobayashi SD, Whitney AR, Voyich JM, Musser JM, Woellner C, Schäffer AA, Puck JM, Grimbacher B (October 2007). “STAT3 mutations in the hyper-IgE syndrome”. N. Engl. J. Med. 357 (16): 1608–19. doi:10.1056/NEJMoa073687. PMID 17881745.
  32. ↑ Ling JC, Freeman AF, Gharib AM, Arai AE, Lederman RJ, Rosing DR, Holland SM (March 2007). “Coronary artery aneurysms in patients with hyper IgE recurrent infection syndrome”. Clin. Immunol. 122 (3): 255–8. doi:10.1016/j.clim.2006.10.005. PMID 17098478.
  33. ↑ Hutto JO, Bryan CS, Greene FL, White CJ, Gallin JI (March 1988). “Cryptococcosis of the colon resembling Crohn’s disease in a patient with the hyperimmunoglobulinemia E-recurrent infection (Job’s) syndrome”. Gastroenterology. 94 (3): 808–12. PMID 3338649.
  34. ↑ O’Connell AC, Puck JM, Grimbacher B, Facchetti F, Majorana A, Gallin JI, Malech HL, Holland SM (February 2000). “Delayed eruption of permanent teeth in hyperimmunoglobulinemia E recurrent infection syndrome”. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 89 (2): 177–85. doi:10.1067/moe.2000.103129. PMID 10673653.
  35. ↑ Tam JS, Routes JM (2013). “Common variable immunodeficiency”. Am J Rhinol Allergy. 27 (4): 260–5. doi:10.2500/ajra.2013.27.3899. PMC 3901442. PMID 23883805.
  36. ↑ Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C (February 2012). “Morbidity and mortality in common variable immune deficiency over 4 decades”. Blood. 119 (7): 1650–7. doi:10.1182/blood-2011-09-377945. PMC 3286343. PMID 22180439.
  37. ↑ Oksenhendler E, Gérard L, Fieschi C, Malphettes M, Mouillot G, Jaussaud R, Viallard JF, Gardembas M, Galicier L, Schleinitz N, Suarez F, Soulas-Sprauel P, Hachulla E, Jaccard A, Gardeur A, Théodorou I, Rabian C, Debré P (May 2008). “Infections in 252 patients with common variable immunodeficiency”. Clin. Infect. Dis. 46 (10): 1547–54. doi:10.1086/587669. PMID 18419489.
  38. ↑ Roifman CM, Rao CP, Lederman HM, Lavi S, Quinn P, Gelfand EW (April 1986). “Increased susceptibility to Mycoplasma infection in patients with hypogammaglobulinemia”. Am. J. Med. 80 (4): 590–4. PMID 3963038.
  39. ↑ Yong PF, Thaventhiran JE, Grimbacher B (2011). ““A rose is a rose is a rose,” but CVID is Not CVID common variable immune deficiency (CVID), what do we know in 2011?”. Adv. Immunol. 111: 47–107. doi:10.1016/B978-0-12-385991-4.00002-7. PMID 21970952.
  40. ↑ Salzer U, Chapel HM, Webster AD, Pan-Hammarström Q, Schmitt-Graeff A, Schlesier M, Peter HH, Rockstroh JK, Schneider P, Schäffer AA, Hammarström L, Grimbacher B (August 2005). “Mutations in TNFRSF13B encoding TACI are associated with common variable immunodeficiency in humans”. Nat. Genet. 37 (8): 820–8. doi:10.1038/ng1600. PMID 16007087.
  41. ↑ Quinti I, Soresina A, Spadaro G, Martino S, Donnanno S, Agostini C, Claudio P, Franco D, Maria Pesce A, Borghese F, Guerra A, Rondelli R, Plebani A (May 2007). “Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency”. J. Clin. Immunol. 27 (3): 308–16. doi:10.1007/s10875-007-9075-1. PMID 17510807.
  42. ↑ Nissenkorn A, Ben-Zeev B (2015). “Ataxia telangiectasia”. Handb Clin Neurol. 132: 199–214. doi:10.1016/B978-0-444-62702-5.00014-7. PMID 26564081.
  43. ↑ Rothblum-Oviatt C, Wright J, Lefton-Greif MA, McGrath-Morrow SA, Crawford TO, Lederman HM (November 2016). “Ataxia telangiectasia: a review”. Orphanet J Rare Dis. 11 (1): 159. doi:10.1186/s13023-016-0543-7. PMC 5123280. PMID 27884168.
  44. ↑ Crawford TO (December 1998). “Ataxia telangiectasia”. Semin Pediatr Neurol. 5 (4): 287–94. PMID 9874856.
  45. ↑ Boder E (1985). “Ataxia-telangiectasia: an overview”. Kroc Found Ser. 19: 1–63. PMID 2415689.
  46. ↑ Hoche F, Seidel K, Theis M, Vlaho S, Schubert R, Zielen S, Kieslich M (June 2012). “Neurodegeneration in ataxia telangiectasia: what is new? What is evident?”. Neuropediatrics. 43 (3): 119–29. doi:10.1055/s-0032-1313915. PMID 22614068.
  47. ↑ BODER E, SEDGWICK RP (April 1958). “Ataxia-telangiectasia; a familial syndrome of progressive cerebellar ataxia, oculocutaneous telangiectasia and frequent pulmonary infection”. Pediatrics. 21 (4): 526–54. PMID 13542097.
  48. ↑ Sahama I, Sinclair K, Pannek K, Lavin M, Rose S (August 2014). “Radiological imaging in ataxia telangiectasia: a review”. Cerebellum. 13 (4): 521–30. doi:10.1007/s12311-014-0557-4. PMID 24683014.
  49. ↑ Lin DD, Barker PB, Lederman HM, Crawford TO (January 2014). “Cerebral abnormalities in adults with ataxia-telangiectasia”. AJNR Am J Neuroradiol. 35 (1): 119–23. doi:10.3174/ajnr.A3646. PMC 4106125. PMID 23886747.
  50. ↑ Nowak-Wegrzyn A, Crawford TO, Winkelstein JA, Carson KA, Lederman HM (April 2004). “Immunodeficiency and infections in ataxia-telangiectasia”. J. Pediatr. 144 (4): 505–11. doi:10.1016/j.jpeds.2003.12.046. PMID 15069401.
  51. ↑ T. Zenone, P. J. Souquet, C. Cunningham-Rundles & J. P. Bernard (1996). “Hodgkin’s disease associated with IgA and IgG subclass deficiency”. Journal of internal medicine. 240 (2): 99–102. PMID 8810936. Unknown parameter |month= ignored (help)
  52. ↑ W. B. Klaustermeyer, M. E. Gianos, M. L. Kurohara, H. T. Dao & D. C. Heiner (1992). “IgG subclass deficiency associated with corticosteroids in obstructive lung disease”. Chest. 102 (4): 1137–1142. PMID 1343817. Unknown parameter |month= ignored (help)
  53. ↑ I. Qvarfordt, G. C. Riise, B. A. Andersson & S. Larsson (2001). “IgG subclasses in smokers with chronic bronchitis and recurrent exacerbations”. Thorax. 56 (6): 445–449. PMID 11359959. Unknown parameter |month= ignored (help)

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Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Epidemiology and Demographics

XLA is an X-linked disorder, and therefore is almost always limited to males. It occurs in a frequency of about 1 in 100,000 male newborns, and has no ethnic predisposition.

References

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Risk Factors

Risk Factors

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References

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Screening

Screening

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References

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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Natural History

Complications

Prognosis

References

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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Serology | Chest X Ray | CT | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

sr:Конгенитална агамаглобулинемија (Брутон) he:XLA de:Agammaglobulinämie

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