454:(GVHD), a subject of ongoing investigation. VSTs have been produced primarily by ex-vivo cultures and by the expansion of T-lymphocytes after stimulation with viral antigens. This is carried out by using donor-derived antigen-presenting cells. These new methods have reduced culture time to 10–12 days by using specific cytokines from adult donors or virus-naive cord blood. This treatment is far quicker and with a substantially higher success rate than the 3–6 months it takes to carry out HSCT on a patient diagnosed with a primary immunodeficiency. T-lymphocyte therapies are still in the experimental stage; few are even in clinical trials, none have been FDA approved, and availability in clinical practice may be years or even a decade or more away.
272:"possible" in the diagnosis of primary immunodeficiency. "Definitive" diagnosis is made when it is likely that in 20 years, the patient has a >98% chance of the same diagnosis being made; this level of diagnosis is achievable with the detection of a genetic mutation or very specific circumstantial abnormalities. "Probable" diagnosis is made when no genetic diagnosis can be made, but the patient has all other characteristics of a particular disease; the chance of the same diagnosis being made 20 years later is estimated to be 85-97%. Finally, a "possible" diagnosis is made when the patient has only some of the characteristics of a disease which are present, but not all.
88:(IEIs) as of 2019, the vast majority of which are PIDs, most are very rare. About 1 in 500 people in the United States are born with a primary immunodeficiency. Immune deficiencies can result in persistent or recurring infections, auto-inflammatory disorders, tumors, and disorders of various organs. There are currently limited treatments available for these conditions; most are specific to a particular type of PID. Research is currently evaluating the use of stem cell transplants (HSCT) and experimental gene therapies as avenues for treatment in limited subsets of PIDs.
36:
271:
Criteria for diagnosis were agreed in 1999. For instance, an antibody deficiency can be diagnosed in the presence of low immunoglobulins, recurrent infections and failure of the development of antibodies on exposure to antigens. The 1999 criteria also distinguish between "definitive", "probable" and
449:
Virus-specific T-lymphocytes (VST) therapy is used for patients who have received hematopoietic stem cell transplantation that has proven to be unsuccessful. It is a treatment that has been effective in preventing and treating viral infections after HSCT. VST therapy uses active donor T-cells that
353:
The treatment of primary immunodeficiencies depends foremost on the nature of the abnormality. Somatic treatment of primarily genetic defects is in its infancy. Most treatment is therefore passive and palliative, and falls into two modalities: managing infections and boosting the immune system.
124:
By definition, primary immune deficiencies are due to genetic causes. They may result from a single genetic defect, but most are multifactorial. They may be caused by recessive or dominant inheritance. Some are latent, and require a certain environmental trigger to become manifest, like the
290:
recognizes nine classes of primary immunodeficiencies, totaling over 120 conditions. A 2014 update of the classification guide added a 9th category and added 30 new gene defects from the prior 2009 version. As of 2019, there are approximately 430 forms of PID that have been identified.
414:
to more specific self-inactivating vector platforms around 2006. The viral vectors randomly insert their sequences into the genomes. However, it is rarely used because of a risk of developing post-treatment T-cell leukemia as a result of interfering
427:
A survey of 10,000 American households revealed that the prevalence of diagnosed primary immunodeficiency approaches 1 in 1200. This figure does not take into account people with mild immune system defects who have not received a formal diagnosis.
435:, are fairly common, with random groups of people (such as otherwise healthy blood donors) having a rate of 1:600. Other disorders are distinctly more uncommon, with incidences between 1:100,000 and 1:2,000,000 being reported.
1399:
Genova E, Cavion F, Lucafò M, Pelin M, Lanzi G, Masneri S, et al. (August 2020). "Biomarkers and
Precision Therapy for Primary Immunodeficiencies: An In Vitro Study Based on Induced Pluripotent Stem Cells From Patients".
394:
which has been in a trial for few immune deficiencies affecting the hematopoietic system. Over the past two decades there were some successful treatments of patients with specific primary immunodeficiencies (PID), including
840:
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)".
96:
The precise symptoms of a primary immunodeficiency depend on the type of defect. Generally, the symptoms and signs that lead to the diagnosis of an immunodeficiency include recurrent or persistent
104:
as a result of infection. Particular organ problems (e.g. diseases involving the skin, heart, facial development and skeletal system) may be present in certain conditions. Others predispose to
80:(PID), the immune deficiency must be inborn, not caused by secondary factors such as other disease, drug treatment, or environmental exposure to toxins. Most primary immunodeficiencies are
963:"Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005"
1607:
457:
Induced pluripotent stem cells obtained reprogramming patients' cells, for example leukocytes, are a promising tool to study these pathologies and develop personalized therapies.
878:"Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency"
2032:
376:(IVIG) or subcutaneous immunoglobulin (SCIG) may be available. Antibiotic prophylaxis is also commonly used to prevent respiratory tract infections in these patients.
84:; the majority are diagnosed in children under the age of one, although milder forms may not be recognized until adulthood. While there are over 430 recognized
1600:
1315:
Porta F, Forino C, De
Martiis D, Soncini E, Notarangelo L, Tettoni K, et al. (June 2008). "Stem cell transplantation for primary immunodeficiencies".
268:
Due to the rarity of many primary immunodeficiencies, many of the above tests are highly specialised and tend to be performed in research laboratories.
1593:
17:
2025:
287:
125:
presence in the environment of a reactive allergen. Other problems become apparent due to aging of bodily and cellular maintenance processes.
2310:
681:"Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee"
1089:"Antibiotic prophylaxis for the prevention of respiratory tract infections in antibody deficient patients: A retrospective cohort study"
1825:
1673:
1480:"International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity"
2018:
1862:
396:
450:
are isolated from alloreactive T-cells which have proven immunity against one or more viruses. Such donor T-cells often cause acute
419:
and because of ethical issues. But the progress in gene therapy is promising for the future of treating primary immunodeficiencies.
116:). The nature of the infections, as well as the additional features, may provide clues as to the exact nature of the immune defect.
443:
2322:
1989:
1691:
432:
2345:
1994:
2279:
479:
Discovery of novel genetic causes of innate immunodeficiencies accelerated greatly in the 2010s due to high-throughput
281:
1929:
1846:
1757:
326:
322:
246:
Tests for B cell function: antibodies to routine immunisations and commonly acquired infections, quantification of
2305:
257:
2317:
1999:
1869:
1741:
576:"Lethal Infectious Diseases as Inborn Errors of Immunity: Toward a Synthesis of the Germ and Genetic Theories"
2340:
2300:
2246:
2097:
1585:
876:
Al-Herz W, Bousfiha A, Casanova JL, Chatila T, Conley ME, Cunningham-Rundles C, et al. (22 April 2014).
1736:
400:
390:
does not exist a causal therapy that would "repair" the mutation. Although there is a therapeutic option,
334:
679:
Tangye SG, Al-Herz W, Bousfiha A, Chatila T, Cunningham-Rundles C, Etzioni A, et al. (January 2020).
321:
is an exception, and is present in 1 in 500 people. Some of the more frequently seen forms of PID include
1849:
1701:
1668:
465:
330:
2350:
2192:
373:
294:
Different forms of PID have different mechanisms. Rough categorizations of conditions divide them into
2375:
1835:
1815:
1730:
1725:
1720:
1715:
1710:
1645:
1478:
Picard C, Bobby Gaspar H, Al-Herz W, Bousfiha A, Casanova JL, Chatila T, et al. (January 2018).
497:
473:
451:
369:
342:
85:
1884:
101:
108:, where the immune system attacks the body's own tissues, or tumours (sometimes specific forms of
1984:
1969:
1965:
1352:"Adoptive immunotherapy for primary immunodeficiency disorders with virus-specific T lymphocytes"
232:
961:
Notarangelo L, Casanova JL, Conley ME, Chapel H, Fischer A, Puck J, et al. (April 2006).
517:
Erjavec SO, Gelfman S, Abdelaziz AR, Lee EY, Monga I, Alkelai A, et al. (February 2022).
168:
Quantification of the different types of mononuclear cells in the blood (i.e. lymphocytes and
1959:
1939:
1660:
1350:
Naik S, Nicholas SK, Martinez CA, Leen AM, Hanley PJ, Gottschalk SM, et al. (May 2016).
1898:
1182:
Cavazzana M, Six E, Lagresle-Peyrou C, André-Schmutz I, Hacein-Bey-Abina S (February 2016).
472:
in 1952. Primary immunodeficiencies were initially classified in 1970 by a committee of the
1979:
1683:
1276:"Population prevalence of diagnosed primary immunodeficiency diseases in the United States"
530:
357:
Reduction of exposure to pathogens may be recommended, and in many situations prophylactic
476:. At the time, they identified 16 immunodeficiencies. By 1998, the number had reached 50.
446:
may be possible for Severe
Combined Immune Deficiency and other severe immunodeficiences.
8:
2010:
591:
209:
189:
534:
49:
Please help update this article to reflect recent events or newly available information.
2250:
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Bucciol G, Moens L, Bosch B, Bossuyt X, Casanova JL, Puel A, Meyts I (February 2019).
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Grammatikos A, Albur M, Gompels M, Barnaby CL, Allan S, Johnston S (October 2020).
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134:
81:
77:
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1921:
1105:
1088:
133:
The basic tests performed when an immunodeficiency is suspected should include a
1367:
1184:"Gene Therapy for X-Linked Severe Combined Immunodeficiency: Where Do We Stand?"
1003:
979:
962:
2178:
1974:
1874:
1696:
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696:
640:
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318:
146:
1615:
1495:
1292:
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2369:
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2131:
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894:
519:"Whole exome sequencing in Alopecia Areata identifies rare variants in KRT82"
70:
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784:
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2167:
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1950:
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1019:
988:
913:
862:
854:
818:
774:
714:
658:
609:
560:
469:
411:
391:
1529:"Lessons learned from the study of human inborn errors of innate immunity"
2255:
2242:
2149:
2087:
1448:
1199:
1146:
781:
468:
was one of the first described primary immunodeficiencies, discovered by
142:
1328:
1181:
932:
has been checked and does not affect the cited material, please replace
2288:
2227:
2197:
2120:
2058:
1955:
1916:
358:
307:
261:
138:
2260:
1413:
362:
253:
97:
2202:
2054:
2042:
1906:
1641:
791:
Grammatikos A, Bright P, Bhatnagar R, Johnston S (September 2020).
387:
240:
169:
113:
790:
1477:
929:
236:
1781:
1650:
960:
483:
technologies. As of 2019, more than 430 have been categorized.
303:
299:
217:
193:
173:
164:
Other tests are performed depending on the suspected disorder:
109:
1086:
875:
622:
1579:
1128:
Booth C, Romano R, Roncarolo MG, Thrasher AJ (October 2019).
623:
Notarangelo LD, Bacchetta R, Casanova JL, Su HC (July 2020).
73:
is missing or does not function normally. To be considered a
1314:
1127:
793:"How to investigate a suspected immune deficiency in adults"
678:
225:
221:
213:
205:
201:
197:
2040:
516:
181:
177:
1526:
1349:
839:
625:"Human inborn errors of immunity: An expanding universe"
1398:
741:"The molecular pathology of primary immunodeficiencies"
1001:
149:
levels (the three most important types of antibodies:
1177:
1175:
1002:
Cooper MA, Pommering TL, Korányi K (November 2003).
738:
372:, immunoglobulin replacement therapy in the form of
27:
Resulting from inborn deficiencies in immune system
1035:
431:Milder forms of primary immunodeficiency, such as
386:For primary immunodeficiencies that are caused by
383:therapies like corticosteroids may be prescribed.
1231:Rabino I (2003). "Gene therapy: ethical issues".
1172:
2367:
1031:
1029:
397:X-linked severe combined immunodeficiency (SCID)
176:(dependent on their cell surface markers, e.g.
1580:International Union of Immunological societies
1533:The Journal of Allergy and Clinical Immunology
1356:The Journal of Allergy and Clinical Immunology
967:The Journal of Allergy and Clinical Immunology
410:Gene therapy evolved in the 90s from using of
288:International Union of Immunological Societies
2026:
1601:
1435:Bruton OC (June 1952). "Agammaglobulinemia".
1026:
1273:
573:
1130:"Gene therapy for primary immunodeficiency"
2033:
2019:
1826:Purine nucleoside phosphorylase deficiency
1674:Transient hypogammaglobulinemia of infancy
1608:
1594:
1036:McCusker C, Warrington R (November 2011).
231:Tests for T cell function: skin tests for
69:are disorders in which part of the body's
1552:
1503:
1375:
1291:
1267:
1207:
1155:
1145:
1104:
1063:
1053:
978:
903:
893:
808:
764:
739:Lim MS, Elenitoba-Johnson KS (May 2004).
734:
732:
730:
728:
726:
724:
704:
648:
599:
550:
2164:
1042:Allergy, Asthma, and Clinical Immunology
1274:Boyle JM, Buckley RH (September 2007).
869:
14:
2368:
2323:Neutrophil-specific granule deficiency
2224:
2117:
1990:Terminal complement pathway deficiency
1434:
1402:Clinical Pharmacology and Therapeutics
1230:
946:|...|checked=yes}}
721:
510:
2014:
1589:
433:selective immunoglobulin A deficiency
91:
2346:Neutrophil immunodeficiency syndrome
995:
745:The Journal of Molecular Diagnostics
674:
672:
670:
668:
592:10.1146/annurev-pathol-031920-101429
574:Casanova JL, Abel L (January 2021).
29:
1995:Paroxysmal nocturnal hemoglobinuria
954:
216:+), as well as activation markers:
24:
1233:Theoretical Medicine and Bioethics
379:In cases of autoimmune disorders,
282:List of primary immunodeficiencies
275:
25:
2387:
1573:
665:
403:and metabolic conditions such as
317:Most forms of PID are very rare.
286:There are many forms of PID. The
2239:Granulocytopenia/agranulocytosis
1758:Common variable immunodeficiency
327:severe combined immunodeficiency
323:common variable immunodeficiency
34:
18:Primary immunodeficiency disease
1930:Idiopathic CD4+ lymphocytopenia
1520:
1471:
1428:
1392:
1343:
1308:
1224:
1121:
1080:
422:
258:nitro blue tetrazolium chloride
2000:Complement receptor deficiency
1870:Adenosine deaminase deficiency
1484:Journal of Clinical Immunology
1280:Journal of Clinical Immunology
1093:Clinical Infection in Practice
833:
685:Journal of Clinical Immunology
616:
567:
13:
1:
2341:Chronic granulomatous disease
2301:Leukocyte adhesion deficiency
2247:Severe congenital neutropenia
2098:Chronic granulomatous disease
757:10.1016/S1525-1578(10)60493-X
503:
233:delayed-type hypersensitivity
1106:10.1016/j.clinpr.2020.100048
1004:"Primary immunodeficiencies"
348:
128:
7:
1669:X-linked agammaglobulinemia
1368:10.1016/j.jaci.2015.12.1311
1317:Bone Marrow Transplantation
980:10.1016/j.jaci.2005.12.1347
486:
466:X-linked agammaglobulinemia
438:
331:X-linked agammaglobulinemia
10:
2392:
2351:Myeloperoxidase deficiency
2193:Hypereosinophilic syndrome
1545:10.1016/j.jaci.2018.07.013
1055:10.1186/1710-1492-7-S1-S11
1038:"Primary immunodeficiency"
810:10.1016/j.rmed.2020.106100
697:10.1007/s10875-019-00737-x
641:10.1126/sciimmunol.abb1662
580:Annual Review of Pathology
543:10.1038/s41467-022-28343-3
460:
374:intravenous immunoglobulin
279:
67:Primary immunodeficiencies
2331:
2287:
2278:
2217:
2157:
2148:
2110:
2073:
2066:
2053:
1938:
1915:
1897:
1845:
1775:
1750:
1682:
1659:
1640:
1631:
1496:10.1007/s10875-017-0464-9
1293:10.1007/s10875-007-9103-1
1008:American Family Physician
498:Inborn errors of immunity
474:World Health Organization
452:graft-versus-host disease
370:humoral immune deficiency
119:
86:inborn errors of immunity
43:This article needs to be
2318:Chédiak–Higashi syndrome
2280:Disorder of phagocytosis
1885:Bare lymphocyte syndrome
1737:Wiskott–Aldrich syndrome
1134:Human Molecular Genetics
925:10.3389/fimmu.2014.00460
895:10.3389/fimmu.2014.00162
401:Wiskott–Aldrich syndrome
335:Wiskott–Aldrich syndrome
264:, bactericidal activity.
1985:Complement 3 deficiency
1970:Complement 4 deficiency
1966:Complement 2 deficiency
1245:10.1023/a:1022967623162
882:Frontiers in Immunology
412:gammaretroviral vectors
256:function: reduction of
172:): different groups of
855:10.1006/clim.1999.4799
444:Bone marrow transplant
417:tumor-suppressor genes
239:and allogeneic cells,
1960:Hereditary angioedema
1816:Ataxia–telangiectasia
1661:Hypogammaglobulinemia
938:|...}}
523:Nature Communications
343:ataxia–telangiectasia
208:and immunoglobulin),
1980:Properdin deficiency
1801:Di George's syndrome
1684:Dysgammaglobulinemia
1449:10.1542/peds.9.6.722
1323:(Suppl 2): S83–S86.
1200:10.1089/hum.2015.137
797:Respiratory Medicine
235:, cell responses to
210:natural killer cells
137:(including accurate
1362:(5): 1498–1505.e1.
1329:10.1038/bmt.2008.61
843:Clinical Immunology
535:2022NatCo..13..800E
243:production by cells
102:developmental delay
2251:Cyclic neutropenia
1832:Hyper IgM syndrome
1742:Hyper-IgE syndrome
1707:Hyper IgM syndrome
1623:disorders causing
1188:Human Gene Therapy
1147:10.1093/hmg/ddz170
629:Science Immunology
106:autoimmune disease
92:Signs and symptoms
2363:
2362:
2359:
2358:
2333:Respiratory burst
2274:
2273:
2270:
2269:
2213:
2212:
2144:
2143:
2140:
2139:
2106:
2105:
2008:
2007:
1893:
1892:
1792:thymic hypoplasia
1777:T cell deficiency
1771:
1770:
1014:(10): 2001–2008.
381:immunosuppression
339:DiGeorge syndrome
82:genetic disorders
64:
63:
16:(Redirected from
2383:
2376:Immunodeficiency
2285:
2284:
2232:
2222:
2221:
2172:
2162:
2161:
2155:
2154:
2125:
2115:
2114:
2081:
2071:
2070:
2064:
2063:
2035:
2028:
2021:
2012:
2011:
1880:ZAP70 deficiency
1811:Nezelof syndrome
1657:
1656:
1638:
1637:
1625:immunodeficiency
1610:
1603:
1596:
1587:
1586:
1567:
1566:
1556:
1524:
1518:
1517:
1507:
1475:
1469:
1468:
1432:
1426:
1425:
1414:10.1002/cpt.1837
1396:
1390:
1389:
1379:
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1295:
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1228:
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1170:
1169:
1159:
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1125:
1119:
1118:
1108:
1084:
1078:
1077:
1067:
1057:
1048:(Suppl 1): S11.
1033:
1024:
1023:
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993:
992:
982:
958:
952:
951:
949:
947:
939:
917:
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897:
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831:
830:
812:
788:
779:
778:
768:
736:
719:
718:
708:
676:
663:
662:
652:
635:(49): eabb1662.
620:
614:
613:
603:
571:
565:
564:
554:
514:
493:Immunodeficiency
388:genetic mutation
365:may be advised.
296:humoral immunity
135:full blood count
78:immunodeficiency
59:
56:
50:
38:
37:
30:
21:
2391:
2390:
2386:
2385:
2384:
2382:
2381:
2380:
2366:
2365:
2364:
2355:
2327:
2266:
2225:
2209:
2165:
2136:
2118:
2102:
2074:
2049:
2039:
2009:
2004:
1941:
1934:
1922:Lymphocytopenia
1920:
1911:
1889:
1865:
1852:
1841:
1797:hypoparathyroid
1779:
1767:
1746:
1678:
1648:
1627:
1614:
1576:
1571:
1570:
1525:
1521:
1476:
1472:
1433:
1429:
1397:
1393:
1348:
1344:
1313:
1309:
1272:
1268:
1229:
1225:
1180:
1173:
1140:(R1): R15–R23.
1126:
1122:
1085:
1081:
1034:
1027:
1000:
996:
959:
955:
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919:(Erratum:
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368:In the case of
351:
310:disorders, and
284:
278:
276:Classifications
212:and monocytes (
131:
122:
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2267:
2265:
2264:
2258:
2253:
2235:
2233:
2219:
2215:
2214:
2211:
2210:
2208:
2207:
2206:
2205:
2200:
2195:
2186:
2179:granulocytosis
2175:
2173:
2159:
2152:
2146:
2145:
2142:
2141:
2138:
2137:
2135:
2134:
2128:
2126:
2112:
2108:
2107:
2104:
2103:
2101:
2100:
2095:
2090:
2084:
2082:
2068:
2061:
2051:
2050:
2038:
2037:
2030:
2023:
2015:
2006:
2005:
2003:
2002:
1997:
1992:
1987:
1982:
1977:
1975:MBL deficiency
1972:
1963:
1946:
1944:
1936:
1935:
1933:
1932:
1926:
1924:
1913:
1912:
1910:
1909:
1903:
1901:
1895:
1894:
1891:
1890:
1888:
1887:
1882:
1877:
1875:Omenn syndrome
1872:
1856:
1854:
1843:
1842:
1840:
1839:
1820:
1819:
1813:
1804:
1787:
1785:
1773:
1772:
1769:
1768:
1766:
1765:
1760:
1754:
1752:
1748:
1747:
1745:
1744:
1739:
1734:
1728:
1723:
1718:
1713:
1704:
1702:IgM deficiency
1699:
1697:IgG deficiency
1694:
1692:IgA deficiency
1688:
1686:
1680:
1679:
1677:
1676:
1671:
1665:
1663:
1654:
1635:
1629:
1628:
1613:
1612:
1605:
1598:
1590:
1584:
1583:
1575:
1574:External links
1572:
1569:
1568:
1539:(2): 507–527.
1519:
1470:
1443:(6): 722–728.
1427:
1408:(2): 358–367.
1391:
1342:
1307:
1286:(5): 497–502.
1266:
1223:
1194:(2): 108–116.
1171:
1120:
1079:
1025:
994:
973:(4): 883–896.
953:
868:
849:(3): 190–197.
832:
780:
720:
664:
615:
566:
508:
507:
505:
502:
501:
500:
495:
488:
485:
481:DNA sequencing
462:
459:
440:
437:
424:
421:
405:leukodystrophy
350:
347:
319:IgA deficiency
280:Main article:
277:
274:
266:
265:
250:
248:IgG subclasses
244:
229:
147:immunoglobulin
130:
127:
121:
118:
93:
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26:
9:
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2293:degranulation
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2176:
2174:
2171:
2169:
2163:
2160:
2156:
2153:
2151:
2147:
2133:
2132:Monocytopenia
2130:
2129:
2127:
2124:
2122:
2116:
2113:
2109:
2099:
2096:
2094:
2093:Histiocytosis
2091:
2089:
2086:
2085:
2083:
2080:
2078:
2072:
2069:
2065:
2062:
2060:
2056:
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2017:
2016:
2013:
2001:
1998:
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1991:
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1983:
1981:
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1961:
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1937:
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1925:
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1918:
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1807:euparathyroid
1805:
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1523:
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1497:
1493:
1490:(1): 96–128.
1489:
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986:
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957:
945:
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931:
926:
922:
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901:
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891:
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883:
879:
872:
864:
860:
856:
852:
848:
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836:
828:
824:
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816:
811:
806:
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798:
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619:
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581:
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562:
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548:
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536:
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528:
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520:
513:
509:
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496:
494:
491:
490:
484:
482:
477:
475:
471:
467:
458:
455:
453:
447:
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436:
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429:
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408:
406:
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393:
389:
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375:
371:
366:
364:
360:
355:
346:
344:
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328:
324:
320:
315:
313:
309:
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297:
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273:
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249:
245:
242:
238:
234:
230:
227:
223:
219:
215:
211:
207:
203:
199:
195:
194:B lymphocytes
192:), groups of
191:
187:
183:
179:
175:
174:T lymphocytes
171:
167:
166:
165:
162:
160:
156:
152:
148:
144:
140:
136:
126:
117:
115:
111:
107:
103:
99:
89:
87:
83:
79:
76:
72:
71:immune system
68:
58:
46:
41:
32:
31:
19:
2226:
2189:Eosinophilia
2184:Neutrophilia
2166:
2150:Granulocytes
2119:
2075:
2047:granulocytes
2041:Diseases of
1951:C1-inhibitor
1949:
1866:
1859:
1822:
1821:
1806:
1796:
1790:
1763:ICF syndrome
1632:
1536:
1532:
1522:
1487:
1483:
1473:
1440:
1436:
1430:
1405:
1401:
1394:
1359:
1355:
1345:
1320:
1316:
1310:
1283:
1279:
1269:
1239:(1): 31–58.
1236:
1232:
1226:
1191:
1187:
1137:
1133:
1123:
1096:
1092:
1082:
1045:
1041:
1011:
1007:
997:
970:
966:
956:
942:{{
934:{{
885:
881:
871:
846:
842:
835:
800:
796:
751:(2): 59–83.
748:
744:
691:(1): 24–64.
688:
684:
632:
628:
618:
583:
579:
569:
526:
522:
512:
478:
470:Ogden Bruton
464:
456:
448:
442:
430:
426:
423:Epidemiology
409:
392:gene therapy
385:
378:
367:
356:
352:
316:
293:
285:
270:
267:
260:, assays of
188:, TCRαβ and
163:
145:counts) and
132:
123:
95:
74:
66:
65:
52:
44:
2256:Eosinopenia
2243:Neutropenia
2088:Monocytosis
2059:macrophages
1823:peripheral:
359:antibiotics
314:disorders.
306:disorders,
298:disorders,
143:granulocyte
2289:Chemotaxis
2198:Basophilia
1956:Angioedema
1942:deficiency
1940:Complement
1917:Leukopenia
1867:autosomal:
1621:complement
1437:Pediatrics
1099:: 100048.
803:: 106100.
529:(1): 800.
504:References
363:antivirals
312:complement
308:phagocytic
262:chemotaxis
252:Tests for
228:(B cells).
139:lymphocyte
112:, such as
98:infections
55:March 2019
2261:Basopenia
2055:Monocytes
2043:monocytes
1860:x-linked:
1465:245180200
1115:228965989
928:. If the
827:221143773
586:: 23–50.
349:Treatment
254:phagocyte
170:monocytes
129:Diagnosis
2370:Category
2203:Bandemia
2168:-cytosis
2077:-cytosis
1907:HIV/AIDS
1899:Acquired
1850:combined
1642:Antibody
1617:Lymphoid
1563:30075154
1514:29226302
1457:14929630
1422:32243572
1386:26920464
1337:18545252
1302:17577648
1261:34605125
1253:12735489
1218:26790362
1166:31297531
1074:22165913
1020:14655810
989:16680902
914:24795713
863:10600329
819:32799060
775:15096561
715:31953710
659:32651211
610:32289233
561:35145093
487:See also
439:Research
241:cytokine
237:mitogens
114:lymphoma
1646:humoral
1633:Primary
1554:6358521
1505:5742601
1377:4860050
1209:4779287
1157:7773329
1065:3245434
944:erratum
936:erratum
930:erratum
905:4001072
888:: 162.
766:1867474
706:7082301
650:7647049
601:7923385
552:8831607
531:Bibcode
461:History
75:primary
45:updated
2228:-penia
2121:-penia
1863:X-SCID
1847:Severe
1582:(IUIS)
1561:
1551:
1512:
1502:
1463:
1455:
1420:
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1374:
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763:
713:
703:
657:
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608:
598:
559:
549:
304:B-cell
300:T-cell
218:HLA-DR
120:Causes
110:cancer
1853:(B+T)
1751:Other
1461:S2CID
1257:S2CID
1111:S2CID
940:with
823:S2CID
190:TCRγδ
2311:LAD2
2306:LAD1
2291:and
2057:and
2045:and
1619:and
1559:PMID
1510:PMID
1453:PMID
1418:PMID
1382:PMID
1333:PMID
1298:PMID
1249:PMID
1214:PMID
1162:PMID
1070:PMID
1016:PMID
985:PMID
910:PMID
859:PMID
815:PMID
771:PMID
711:PMID
655:PMID
606:PMID
557:PMID
302:and
226:CD80
222:CD25
214:CD14
206:CD21
202:CD20
198:CD19
186:CD3+
182:CD8+
178:CD4+
157:and
141:and
1549:PMC
1541:doi
1537:143
1500:PMC
1492:doi
1445:doi
1410:doi
1406:108
1372:PMC
1364:doi
1360:137
1325:doi
1288:doi
1241:doi
1204:PMC
1196:doi
1152:PMC
1142:doi
1101:doi
1097:7–8
1060:PMC
1050:doi
975:doi
971:117
921:doi
900:PMC
890:doi
851:doi
805:doi
801:171
761:PMC
753:doi
701:PMC
693:doi
645:PMC
637:doi
596:PMC
588:doi
547:PMC
539:doi
361:or
161:).
159:IgM
155:IgA
151:IgG
100:or
2372::
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