Download a case of hashimoto`s thyroiditis with autoimmune haemolytic anaemia

CASE REPORT
A CASE OF HASHIMOTO’S THYROIDITIS WITH AUTOIMMUNE
HAEMOLYTIC ANAEMIA
Debabrata Goswami1, Bhaskar Brahma2,A. B. Ahmed3, Tribeni Sarma4, Basabendra Choudhary5
HOW TO CITE THIS ARTICLE:
DebabrataGoswami, Bhaskar Brahma, A. B. Ahmed, TribeniSarma, BasabendraChoudhary.“A Case of
Hashimoto’s Thyroiditis with Autoimmune Haemolytic Anaemia”.Journal of Evidence based Medicine and
Healthcare; Volume 1, Issue 14, December 08, 2014; Page: 1583-1588.
ABSTRACT: Hashimoto’s thyroiditis is a part of the spectrum of autoimmune thyroid disease and
it is found to be associated with other autoimmune diseases like Addison’s disease, vitiligo, type1
diabetes mellitus, alopecia areata, pernicious anaemia etc. However its association with
autoimmune haemolytic anaemia is uncommon. We reported a case of Hashimoto’s thyroditis
associated with haemolytic anaemia who responded with thyroxine replacement and oral
prednisolone therapy.
KEYWORDS:Hashimoto’s thyroiditis, Auotoimmunehaemolytic anaemia, Thyroxine, Prednisolone.
INTRODUCTION: Autoimmune thyroid diseases comprise a series of interrelated conditions
including hyperthyroid Grave’s disease, Hashimoto's thyroiditis, atrophic autoimmune
hypothyroidism, postpartum thyroiditis, and thyroid-associated ophthalmopathy. These
manifestations can occur synchronously and most frequently as a combination.[1]
Hashimoto's disease is the most common cause of goitrous hypothyroidism in areas of
iodine sufficiency. The mean incidence in women is in the order of 3.5 cases per 1000 people per
year and in men is 0.8 case per 1000 people per year.[2,3,4] The annual risk of developing clinical
hypothyroidism is about 4% when subclinical hypothyroidism is associated with positive TPO
(thyroid peroxidase) antibodies.[2,3,4]
Autoimmune hypothyroidism may be associated with signs or symptoms of other
autoimmune diseases, particularly vitiligo, pernicious anemia, Addison's disease, alopecia areata,
and type 1 diabetes mellitus. Less-common associations include celiac disease, dermatitis
herpetiformis, chronic active hepatitis, rheumatoid arthritis, systemic lupus erythematosus (SLE),
myasthenia gravis, and Sjögren's syndrome.[5] However, not many cases of Hashimoto’s disease
have been found to be associated with autoimmune hemolytic anemia(AIHA). Except for
countries where malaria is endemic, AIHA is the most common form of acquired hemolytic
anemia.[6,7]
CASE REPORT: A 15 year old boy presented with the complaints of gradually progressive,
painless swelling in the front of the neck for 2years, gradually progressive exertional
breathlessness and easy fatiguability for the last 3 months without any history of fever, cough,
palpitation, blackout, joint pain, rash, photosensitivity. He also complained of constipation and
decreased concentration in studies for the last few months. The patient was admitted in a local
hospital 2 months back where he was treated for severe generalized weakness with two units of
red blood transfusion. On examination the boy with an average build, fair nutrition, lusterless hair
and dry, rough skin especially over the palms and soles, was found to have pallor, mild icterus
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CASE REPORT
and mild facial puffiness. His pulse rate was 84 beats per minute, regular in rhythm, normal in
volume and character; all peripheral pulses were palpable equally on both sides. His blood
pressure was 124/ 86 mm Hg. He had a firm, non-tender, diffuse thyroid swelling of size 7 cmx3
cm, with a smooth surface without any pulsation, local rise in temperature. There was mild, nontender, firm splenomegaly on abdominal examination. The deep tendon jerks were slightly
diminished in all the four limbs with delayed relaxation of the ankle jerks on both sides. There
was no evidence of heart failure clinically. Investigations revealed hemoglobin level of4.0 gm%,
total leucocyte count 5160/cumm, platelet count 1.08 lacs/cu mm, ESR 150mm. Reticulocyte
count was 3%, MCV was high, peripheral blood smear showed mixed population of
normochromic, normocytic and hypochromic, microcytic RBCs, anisocytosis, polychromasia, no
hemoparasite. His serum iron was 94mcg/dl, TIBC 331µg/dl and serum ferritin was 118µg/l.
Serum bilirubin was 2.91mg/dl, all unconjugated, AST 198U/L, ALT 81U/L, ALP 53 IU/L, albumin
4.5gm/dl, LDH 2032mg/dl., Serum creatinine 1.2 mg/dl, blood sugar 90 mg/dl and Urine R/E was
positive for hemoglobin. His serum TSH: 300mIU/ml, free T3: 1.33pmol/l, free T4: 3.26pmol/l
and anti TPO antibodies was positive (>1000).Direct Coomb’s test (gel card method) was
positive. G6PD and Hemoglobin electrophoresis were normal, ECG and 2D-Echocardiographywere
normal and Pf/Pv, HBsAg, antiHCV, HIV, VDRL, was non-reactive. Ultrasound thyroid was
suggestive of Hashimoto’s thyroiditis (diffuse increase in bulk, coarse septations, no nodules, and
few prominent lymph nodes near both the poles).
Picture of USG Thyroid
The patient with a diffuse, non-tender goiter, markedly elevated serum TSH, low free T3
and T4 levels was diagnosed as a case of primary hypothyroidism. Raised antiTPO levels and USG
thyroid strongly suggested that the hypothyroidism was due to Hashimoto’s thyroiditis. On the
other hand, the presence of anaemia, unconjugated hyperbilirubinemia, splenomegaly with high
reticulocyte count, PBS suggestive of hemolytic picture, elevated serum LDH and a positive direct
Coomb’s test pointed towards autoimmune hemolytic anemia. The patient was started on oral
Prednisolone 50 mg/day, levothyroxine 75mcg/day and 5 units of packed red cell transfusion.
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Within 4-5 days of starting treatment with oral steroids and thyroxine, patient showed significant
improvement with rise in the hemoglobin level, disappearance of hemoglobinuria and jaundice.
DISCUSSION: Autoimmune diseases comprise of a heterogeneous group of disorders and are
sometimes defined as a clinical syndrome caused by alterations in the immune system such as
activation of T cells or B cells or both, resulting in a spectrum of diseases that can target specific
organs or affect the body systemically.[8]The initiation of autoimmune events in Hashimoto's
thyroiditis may be caused by a molecular mimicry mechanism, abnormal antigen-specific
induction of T cells due to abnormal human leukocyte antigen (HLA)-related genes, mutation of T
cells to form abnormal clones, or an immune defect causing reduced induction of T-suppressor
cells by specific antigens.[9]The detection of anti-thyroid peroxidase (TPO) is one of the reliable
diagnostic tests for Hashimoto's thyroiditis (HT) which was significant in our patient.[10]
The diagnostic features of autoimmune hemolytic anemia include a combination of clinical
and laboratory signs of RBC hemolysis, together with the detection of autoantibodies, as
represented mostly by a positive direct antiglobulin test; which has been demonstrated in our
case. The formation of autoantibodies in autoimmune hemolytic anemia (AIHA) may be due to
the break down in T-cell regulation of B cells, with the emergence of a hostile clone of
immunocytes, or to a change in the structure of the antigen on the patient's red cells, which is
then recognized as non-self by its immune system.[11] Once a red cell is coated by antibody, it will
be destroyed by one or more mechanisms. In most cases the Fc portion of the antibody will be
recognized by the Fc receptor of macrophages, and this will trigger erythrophagocytosis in the
spleen, liver, and bone marrow i.e., extravascular hemolysis. In some cases, the nature of the
antibody (usually an IgM antibody) is such that the antigen-antibody complex on the surface of
red cells is able to activate complement (C). As a result, a large amount of membrane attack
complex will form, and the red cells may be destroyed directly; this is known as intravascular
hemolysis.
Thus due to abnormal immune response, which may be both cell-mediated and antibody
mediated, due to some genetic and or environmental factors, a person may develop both
autoimmune thyroidits and autoimmune hemolytic anemia over time as did the above patient.
However not many such cases have been reported. Interestingly, Hashimoto’s thyroiditis is more
common in females and that too in a much older age group unlike the above 15 year old boy.
It is however very important to identify both the conditions if present in a patient as each
demand a separate line of therapy. Hashimotos thyroiditis which usually has a insidious course
can be treated with daily oral thyroid hormone replacement only. Whereas AIHA, the onset of
which is very often abrupt with the hemoglobin level dropping, within days, to as low as 4 g/dL
needs prompt immune suppression with prednisolone.
Though not common AIHA should always be suspected in any case of Hashimoto’s
thyroiditis especially in presence of abruptly developing and rapidly deteriorating refractory
anemia.
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REFERENCES:
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United States population (1988 to 1994): National Health and Nutrition Examination Survey
(NHANES III). J ClinEndocrinolMetab 2002; 87:489-499.
3. Tunbridge WMG, Evered DC, Hall R, et al: The spectrum of thyroid disease in the
community: the Whickham Survey. ClinEndocrinol 1977; 7:481-493.
4. Vanderpump MP, Tunbridge WM, French JM, et al: The incidence of thyroid disorders in the
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43:55-68.
5. Zeitlin AA et al: Genetic developments in autoimmune thyroid disease: an evolutionary
process. ClinEndocrinol 68:671, 2008[PMID: 18081880]
6. Dacie J: The Haemolytic Anaemias. London, Churchill Livingstone, 1985–1995.
7. Lechner K, Jager U: How I treat autoimmune hemolyticanemias in adults. Blood 116:1831,
2010[PMID: 20548093].
8. Davidson A, Diamond B. Autoimmune diseases. N Engl J Med 2001:345:340-50.
9. Chistiakov DA. Immunogenetics of Hashimoto's thyroiditis. J Autoimmune Dis 2005;2:1-21.
10. KalyanKoti,Rayapa
Reddy
Thumma,SwathanthraNagarajan,AtchyutaMathiHashimoto's
thyroiditis associated Evans syndrome: A rare case report on the clustered autoimmune
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11. Gehrs BC, Friedberg RC. Autoimmune HemolyticAnemia. Am J Hematol 2002;69:258-71.
AUTHORS:
1. DebabrataGoswami
2. Bhaskar Brahma
3. A. B. Ahmed
4. TribeniSarma
5. BasabendraChoudhary
PARTICULARS OF CONTRIBUTORS:
1. Professor & HOD, Department of Medicine,
Gauhati Medical College and Hospital,
Guwahati, Assam.
2. Registrar, Department of Medicine, Gauhati
Medical College and Hospital, Guwahati,
Assam.
3. Assistant Professor, Department of
Medicine, Gauhati Medical College and
Hospital, Guwahati, Assam.
4. Associate Professor, Department of
Medicine, Gauhati Medical College and
Hospital, Guwahati, Assam.
5. Post Graduate Trainee, Department of
Medicine, Gauhati Medical College and
Hospital, Guwahati, Assam.
NAME ADDRESS EMAIL ID OF THE
CORRESPONDING AUTHOR:
Dr. Bhaskar Brahma,
Department of Medicine,
GMCH, Bhangagarh,
Guwahait-32,
Assam.
E-mail: [email protected]
Date
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Date
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Submission: 12/11/2014.
Peer Review: 13/11/2014.
Acceptance: 18/11/2014.
Publishing: 24/11/2014.
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