The Journal of the Arkansas Medical Society Med Journal May 2019 Final 2 - Page 21

Extensive laboratory workup to rule out infectious etiology, fat malabsorption, celiac disease, and inflammatory bowel disease was negative. ferent time periods. Regular treatment with IVIG to target adequate serum levels has shown to be of benefit in reducing morbidity. Patients with thy- moma should be regularly followed and physicians should have a low threshold for testing for immu- nodeficiencies in these patients. Medical Board Legal Issues? REFERENCES isolated. Many autoimmune disorders like PRCA, Myasthenia gravis, Sweet’s syndrome, Diabetes mellitus, primary sclerosing cholangitis (PSC), and ulcerative colitis (UC) have been described in association with GS. 6,8 Numerous hematologic abnormalities including aplastic anemia, hemolytic anemia, macrocytic anemia, and myelodysplastic syndromes are also reported in these patients. 2 Diagnosis of GS requires a high clinical sus- picion. All patients with thymoma or anterior me- diastinal mass should be tested for underlying immunodeficiency. Presence of recurrent sino- pulmonary infections, opportunistic infections, or reduced/absent B cells in these patients should prompt physicians for association with GS. Im- munoglobulin values and flow-cytometric analy- sis of B and T cell subsets should be performed initially. Even if the initial levels are normal, the tests should be repeated every two years as they can develop immunodeficiency in the long run. Thymectomy should be considered in pa- tients with thymoma. Depending on the extent of disease, concurrent chemo and or radiation should be offered. However, thymectomy is inef- fective in reverting back immune deficiency or might even worsen hypogammaglobulinemia. 7,9 IVIG should be provided to all patients in doses necessary to maintain adequate level of serum immunoglobulins. Review of previous cases has shown beneficial effect of IVIG in decreasing hospitalizations and achieve better control of in- fections. 7 In our patient, the diarrhea responded promptly to administration of IVIG. Prognosis in Good’s syndrome is thought to be worse than other immunodeficiencies. Reported survival rate is 70% at five years and 33% at 10 years. 10 CONCLUSION Good’s syndrome can be an underlying etiol- ogy of chronic diarrhea, recurrent sino-pulmonary, or atypical infections. It affects both cell-mediated and humoral immunity. Diagnosis can be challeng- ing and needs high clinical suspicion as thymoma and onset of immunodeficiency can occur at dif- 1. Good RA. Agammaglobulinemia—a provocative experiment of nature. Bulletin of the University of Minnesota 1954;26:1–19. 2. Kelleher P, Misbah SA. Review: What is Good’s syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol Jan. 2003;56(1):12–16. 3. Souadjian JV, Enriquez, P, Silverstein MN, et al. The spectrum of diseases associated with thy- moma. Coincidence or syndrome. Arch Intern Med 1974;134: 374-79. Call Pharmacist/Attorney Darren O’Quinn 1-800-455-0581 4. Kelesidis T, Yang O. Review: Good’s syndrome remains a mystery after 55 years: A systematic review of the scientific evidence. Clinical Im- munology Jun. 2010;135(3):347–363. 5. Oritani K, Medina KL, Tomiyama Y, et al. Limitin: an interferon-like cytokine that preferentially influences B-lymphocyte precursors. Nat Med Immunol. 2000; 3:659–666. 6. Peña C, Intriago M, Muñoz P, Gray AM, Cabrera ME; Association of Good syndrome with Pure Red cell aplasia: A report of one case. Rev Med Chil. 2012 Aug;140(8):1050-2. doi: 10.4067/ S0034-98872012000800013. Review. Span- ish. Little Rock, Arkansas Put your business or service in the hands of 4,400 Arkansas physicians. 7. Tarr PE, Sneller MC, Mechanic LJ, et al. In- fections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. Medicine 2001;80:123–33. 8. B.W. Kirk, S.O. Freedman, Hypogammaglobu- linemia, thymoma and ulcerative colitis, Can. Med. Assoc. J. 96 (1967) 1272–1277 9. Atsushi Kitamura, Naobumi Tochigi, Koichiro Tat- sumi et al; Durable Hypogammaglobulinemia Associated with Thymoma (Good Syndrome); Internal Medicine, 2009;48(19):1749-52. Epub 2009 Oct 1. 10. Hermaszewski RA, Webster AD. Primary hypo- gammaglobulinemia: a survey of clinical mani- festations and complications. Q J Med 1993; 86: 31-42. For more advertising information, contact Penny Henderson at 501.224.8967 or NUMBER 11 MAY 2019 • 261