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1196 Part IX: Lymphocytes and Plasma Cells Chapter 78: Lymphocyte and Plasma Cell Disorders 1197
TABLE 78–1. Classification of Disorders of Lymphocytes and Plasma Cells (Continued )
12. Serum sickness 79 C. T-lymphocyte dysfunction or depletion associated with
13. T-cell lymphocytosis associated with thymoma systemic disease
(Chap. 79) 1. B-cell chronic lymphocytic leukemia (Chap. 92)
14. Toxoplasma gondii mononucleosis (Chap. 82) 2. Hodgkin lymphoma (Chap. 97)
15. Trypanosoma cruzi 80 3. Leprosy 82
16. Viral infectious lymphocytosis (Chap. 79) 4. Lupus erythematosus 83
17. Cat-scratch and other chronic bacterial 5. Sjögren syndrome 84
infection 81 6. Sarcoidosis 85
organisms associated with formation of immune granulomas. Th2- REFERENCES
type CD4+ Th cells, on the other hand, appear better suited to induce
B-cell responses to antigen and direct the immune response against 1. International Union of Immunological Societies Expert Committee on Primary I;
parasitic infestations (Chap. 76). Deficiency or defects in CD4+ regula- Notarangelo LD, Fischer A, et al: Primary immunodeficiencies: 2009 update. J Allergy
Clin Immunol 124:1161–1178, 2009.
tor T cells (T REGs ) can result in autoimmune disease, whereas depletion 2. Conley ME, Dobbs AK, Farmer DM, et al: Primary B cell immunodeficiencies: Com-
or deficiency of Th17 cells can result in impaired resistance to oppor- parisons and contrasts. Annu Rev Immunol 27:199–227, 2009.
tunistic infection (Chap. 76). Depletion of CD4+ T cells in patients 3. Ballow M: Primary immunodeficiency disorders: Antibody deficiency. J Allergy Clin
Immunol 109:581–591, 2002.
infected with human immunodeficiency virus accounts in large part 4. Kyrtsonis MC, Mouzaki A, Maniatis A: Mechanisms of polyclonal hypogammaglobuli-
for the acquired immune deficiency that develops in patients infected naemia in multiple myeloma (MM). Med Oncol 16:73–77, 1999.
with the virus (Chap. 81). 5. Pritsch O, Maloum K, Dighiero G: Basic biology of autoimmune phenomena in chronic
lymphocytic leukemia. Semin Oncol 25:34–41, 1998.
T lymphocytes within a marrow allograft are responsible for initia- 6. Halfdanarson TR, Litzow MR, Murray JA: Hematologic manifestations of celiac dis-
tion of the graft-versus-host disease (GVHD) (Chap. 23). Acute GVHD ease. Blood 109:412–421, 2007.
can lead to severe dermatitis, gastroenteritis, and hepatitis. Chronic 7. Conley ME, Rohrer J, Minegishi Y: X-linked agammaglobulinemia. Clin Rev Allergy
Immunol 19:183–204, 2000.
GVHD can encompass a collage of connective tissue diseases, such as 8. Schiff C, Lemmers B, Deville A, et al: Autosomal primary immunodeficiencies affecting
scleroderma, xerophthalmia, xerostomia, and pulmonary insufficiency, human bone marrow B-cell differentiation. Immunol Rev 178:91–98, 2000.
specifically bronchiolitis obliterans. Eosinophilia, hypergammaglobu- 9. Conley ME, Dobbs AK, Quintana AM, et al: Agammaglobulinemia and absent B lineage
cells in a patient lacking the p85alpha subunit of PI3K. J Exp Med 209:463–470, 2012.
linemia, development of autoantibodies, and plasmacytosis can occur. 10. Sneller MC, Strober W, Eisenstein E, et al: NIH conference. New insights into common
Infection with classic or opportunistic pathogens is a common compli- variable immunodeficiency. Ann Intern Med 118:720–730, 1993.
cation of both acute and chronic GVHD. A similar qualitative reaction, 11. Dalal I, Reid B, Nisbet-Brown E, Roifman CM: The outcome of patients with hypogam-
albeit more limited, is seen in mononucleosis resulting from Epstein- maglobulinemia in infancy and early childhood. J Pediatr 133:144–146, 1998.
Barr virus infection (Chap. 82). 12. Payne M, Hickson ID: Genomic instability and cancer: Lessons from analysis of Bloom’s
syndrome. Biochem Soc Trans 37:553–559, 2009.
13. Di WL, Mellerio JE, Bernadis C, et al: Phase I study protocol for ex vivo lentiviral gene
therapy for the inherited skin disease, Netherton syndrome. Hum Gene Ther Clin Dev
COMBINED T- AND B-CELL DISORDERS 14. Louis AG, Gupta S: Primary selective IgM deficiency: An ignored immunodeficiency.
24:182–190, 2013.
Clin Rev Allergy Immunol 46:104–111, 2014.
Combined T- and B-cell deficiency and dysfunction can result in het- 15. Buchbinder D, Nugent DJ, Fillipovich AH: Wiskott-Aldrich syndrome: Diagnosis, cur-
erogeneous group of disorders that affect both cellular and humoral rent management, and emerging treatments. Appl Clin Genet 7:55–66, 2014.
immunity. The disorder can be severe (severe combined immune 16. Yel L: Selective IgA deficiency. J Clin Immunol 30:10–16, 2010.
deficiency [SCID]) or mild depending on whether the defect is par- 17. Latiff AH, Kerr MA: The clinical significance of immunoglobulin A deficiency. Ann
Clin Biochem 44:131–139, 2007.
tial (hypomorphic defects) or complete (null or amorphic defects). 18. Schroeder HW Jr, Schroeder HW 3rd, Sheikh SM: The complex genetics of common
Complete defects can result in early death, typically during the first variable immunodeficiency. J Investig Med 52:90–103, 2004.
year of life, from uncontrolled infection. An extreme form of SCID 19. Davies EG, Thrasher AJ: Update on the hyper immunoglobulin M syndromes. Br J
Haematol 149:167–180, 2010.
is the T-cell–negative, B-cell–negative, NK-cell–positive phenotype 20. Lanzi G, Ferrari S, Vihinen M, et al: Different molecular behavior of CD40 mutants
with children presenting early in life with severe infections, failure causing hyper-IgM syndrome. Blood 116:5867–5874, 2010.
to thrive, and low-to-absent T-and B-cell numbers and function. 21. Klasen IS, Goertz JH, van de Wiel GA, et al: Hyper-immunoglobulin A in the hyperim-
munoglobulinemia D syndrome. Clin Diagn Lab Immunol 8:58–61, 2001.
Advances in gene therapy and allogeneic stem cell transplantation 22. Bermejo JF, Carbone J, Rodriguez JJ, et al: Macroamylasaemia, IgA hypergammaglob-
offer hope in some cases (Chap. 80). ulinaemia and autoimmunity in a patient with Down syndrome and coeliac disease.
Scand J Gastroenterol 38:445–447, 2003.
23. Drenth JP, Haagsma CJ, van der Meer JW: Hyperimmunoglobulinemia D and periodic
NATURAL KILLER CELL DISORDERS fever syndrome. The clinical spectrum in a series of 50 patients. International Hyper
-IgD Study Group. Medicine (Baltimore) 73:133–144, 1994.
24. Stoffels M, Simon A: Hyper-IgD syndrome or mevalonate kinase deficiency. Curr Opin
Chronic NK cell lymphocytosis is a rare proliferative disorder that can Rheumatol 23:419–423, 2011.
be distinguished from NK cell leukemia and lymphoma by its indolent 25. Korppi M, Van Gijn ME, Antila K: Hyperimmunoglobulinemia D and periodic fever
nature (Chap. 77). Patients typically have neutropenia, anemia, vas- syndrome in children. Review on therapy with biological drugs and case report. Acta
Paediatr 100:21–25, 2011.
culitic syndromes, fever of unknown origin, constitutional symptoms, 26. Yoshimura K, Wakiguchi H: Hyperimmunoglobulinemia D syndrome successfully
cutaneous lesions and autoimmune disorders, including rheumatoid treated with a corticosteroid. Pediatr Int 44:326–327, 2002.
arthritis, Sjögren syndrome, and/or polymyalgia rheumatica. Studies 27. Yong PF, Freeman AF, Engelhardt KR, et al: An update on the hyper-IgE syndromes.
Arthritis Res Ther 14:228, 2012.
seeking to define this condition as a clonal disorder using X-linked gene 28. Burastero SE, Paolucci C, Breda D, et al: Immunological basis for IgE hyper-production
analysis have not yielded consistent findings (Chap. 77). in enfuvirtide-treated HIV-positive patients. J Clin Immunol 26:168–176, 2006.
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