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.
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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
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2. Kelleher P, Misbah SA. Review: What is Good’s
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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.
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4. Kelesidis T, Yang O. Review: Good’s syndrome
remains a mystery after 55 years: A systematic
review of the scientific evidence. Clinical Im-
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5. Oritani K, Medina KL, Tomiyama Y, et al. Limitin:
an interferon-like cytokine that preferentially
influences B-lymphocyte precursors. Nat Med
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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.
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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-
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86: 31-42.
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