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Case report
DOI: 10.5958/2319-5886.2015.00184.8
Open access
COLD AGGLUTININ INDUCED HEMOLYTIC ANEMIA IN A PATIENT WITH

PULMONARY TUBERCULOSIS
1
Lohmror Anurag , Choudhary Richa
ARTICLE INFO
ABSTRACT
th
Received: 30 June 2015

th
Revised: 10 July 2015
nd
Accepted: 2 Sep 2015
1
Senior Resident,
Department of Medicine, Sawai Man
Singh Medical College, Jaipur, India
2
Senior Resident, Department of
Paediatrics, Sawai Man Singh Medical
College, Jaipur, India
Authors details:
author:
Lohmror
Anurag
Senior Resident, Department of
Medicine, Sawai Man Singh Medical
College, Jaipur, India
Email: [email protected]
Corresponding
Autoimmune hemolytic anemias (AIHA) are an uncommon group of disorders

characterized by red cell destruction due to autoantibodies. Though usually
idiopathic, AIHA is commonly associated with lymphoproliferative disorders,
infections, autoimmune disease, and some drugs. This report describes a case
of 25 year old female presenting history of fever associated with cough and
fatigue. There was a past history of receiving blood transfusion on four
occasions. The HRCT thorax demonstrated fine nodular densities in right upper
lobe, suggestive of tuberculosis. Abdominal ultrasonography revealed mild
splenomegaly. A bone marrow biopsy performed on the patient revealed
erythroid hyperplasia. There was no evidence of any malignancy. Diagnosis of
cold autoantibody hemolytic anemia complicated by pulmonary tuberculosis was
made. The patient was managed with blood transfusions and treated with antitubercular agents. The occurrence of AIHA in pulmonary tuberculosis is rare.
Autoimmune hemolytic
anemia, Tuberculosis, Cold agglutinin
Keywords:
INTRODUCTION
Autoimmune hemolytic anemia (AIHA) occurs when a
patient produces pathologic antibodies that attach to and
lead to the destruction of their RBCs with consequent
anemia. AIHA can be classified as warm AIHA and cold
AIHA according to the characteristic temperature activity of
[1]
the antibodies . Occasionally, a patient may have mixed
cold and warm active antibodies. Primary (idiopathic) AIHA
[2]
is less frequent than secondary AIHA . Autoimmune
antibodies, particularly cold-reactive antibodies, are
sometimes produced following an infection or immune
[3,4,5,6]
defects or lympho proliferative disorders or drugs
.
These secondary cases are often challenging since not
only AIHA, but also the underlying disease must be
diagnosed and treated. Association of autoimmune
haemolytic anaemia with pulmonary tuberculosis has been
seldom reported. Tuberculosis being a common disease,
the association with hemolytic anemia should be
recognized and treated judiciously.
CASE REPORT
A 25 year old female presented to our department with a
history of fever associated with cough and fatigue. Physical
examination revealed pallor and mild splenomegaly. Blood
pressure, pulse rate and temperature were within normal
limits. Bilateral crepitations could be heard on chest
auscultation. Other findings on physical examination were
unremarkable. There was a past history of receiving blood
transfusion on four occasions. The laboratory investigations
demonstrated severe anemia (Hemoglobin 4.5 g/dl; MCV
74.3fl MCH 18.4pg MCHC 24.7g/dl) with a normal white
blood count and platelet count. The peripheral blood smear

showed
microcytic
hypochromic
RBCs
with
anisopoikilocytosis, elliptocytes, tear drop cells, target cells.
The reticulocyte count was 5.82% and reticulocyte index
was calculated to be 2.0. Serum LDH was raised (991 U/L).
The Direct Coombs test was positive with anti-C3d
specificity; anti-IgG was negative. The cold agglutinin titre
was 1:256. The patient tested negative for anti nuclear
antibodies and ds-DNA antibodies, HIV, Hepatitis B surface
antigen and anti HCV antibodies. Sputum was positive for
acid fast bacilli. Chest X-ray showed diffuse small nodular
infiltrates over right lung fields. The HRCT thorax
demonstrated fine nodular densities in right upper lobe,
suggestive of tuberculosis. Abdominal ultrasonography
revealed mild splenomegaly. A bone marrow biopsy
performed on the patient revealed erythroid hyperplasia.
There was no evidence of any malignancy. Based on the
characteristics discussed in the preceding paragraphs and
available literature, a diagnosis of cold autoantibody
hemolytic anemia complicated by pulmonary tuberculosis
was made.
The patient was managed with blood
transfusions and treated with anti-tubercular agents.
DISCUSSION
Autoimmune hemolytic anemia (AIHA) is a rare disease. In
[7]
a recent population based study
the incidence was
[8]
0.8/100.000/year, but the prevalence is 17/100.000 . There
are two main types of autoimmune hemolytic anemia: warm
antibody and cold antibody induced hemolytic anemia
911
Anurag et al.,
Int J Med Res Health Sci. 2015;4(4):911-912
based on the ability of the autoantibodies to attach to and

destroy red blood cells at different temperatures.
Cold active autoantibodies have the capability to
agglutinate red blood cells at temperatures well below the
normal body temperatures, fix complement, and lead to
immediate intravascular RBC destruction or hepatic
mediated clearance. Two different clinical syndromes are
manifested from cold auto immune antibodies. Cold
Agglutinin Disease is associated with IgM antibodies
usually directed at the RBC I antigen. The responsible
pathologic IgM antibodies are distinguished from naturally
occurring cold autoantibodies by their titre and thermal
amplitude. Natural cold autoantibodies occur in titres less
than 1:64 at 4 C and have no activity at temperatures much
higher than that. However, pathologic cold agglutinins
typically have titres well over 1:1000 and may react at 28[1]
31 C or even up to 37 C . An IgG type of cold reactive
autoantibody with anti P specificity, known as Donath
Landsteiner antibody, characterizes Paroxysmal Cold
Hemoglobinuria.
In contrast, warm active antibodies are typically IgG, may or
may not fix complement, and primarily lead to RBC loss by
splenic removal of sensitized cells. Both CAD and PCH are
less common than warm AIHA and make up approximately
[1]
20% or less of AIHAs.
Almost all cases of cold AIHA in adults seem to be
secondary. The underlying conditions in most cases are
lymphoproliferative diseases, less commonly autoimmune
diseases(SLE) or infections(infectious mononucleosis,
Mycoplasma pneumonia, advanced HIV infections) , and
[3,4,5,6]
rarely drugs
.In some of the cases , the etiology
remains obscured labeling them as primary or idiopathic.
The key component to treatment remains avoidance of
exposure to cold and management of underlying infectious
or malignant process. In contrast to warm AIHA, cold AIHA
[2]
does not respond well to steroids and/or splenectomy .
Transfusion of red cells in AIHA can result in rapid in vivo
destruction of transfused cells due to the presence of auto
antibodies, hence it is of transient benefit, but may be
[2]
required initially in managing severe anemia.
In tuberculosis, hematologic abnormalities like anemia are
common. Possible mechanisms include nutritional
deficiency, malabsorption syndrome, marrow suppression,
[9]
and failure of iron utilization . However, the association of
immune hemolytic anemia with tuberculosis is relatively
[10]
rare . The majority of the cases reported in literature are
disseminated or extra pulmonary tuberculosis.
In our patient with cold autoantibody induced hemolytic
anemia, the work up for secondary causes of AIHA like
lymphoproliferative disorders and SLE was negative, and
tuberculosis was diagnosed on the basis of isolation of
organism in sputum and clinical and radiological findings.
The patient responded to anti tubercular treatment, steroids
were not given.
CONCLUSION
Although rare, pulmonary tuberculosis may be associated
with autoimmune hemolytic anemia. Tuberculosis should be
considered as a differential diagnosis of the etiology of
secondary AIHA because administration of steroids alone
to treat AIHA in such untreated tuberculosis cases may be
detrimental to the patient.
ACKNOWLEDGEMENT: none
Conflict of Interest: Nil
REFERENCES
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Greer JP, Foerster J, editors. Wintrobes Clinical
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Autoimmune hemolytic anemia during adalimumab
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8. Eaton WW, Rose NR, Kalaydjian A, Pedersen MG,
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912
Anurag et al.,
Int J Med Res Health Sci. 2015;4(4):911-912

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Available online at: www.ijmrhs.

COLD AGGLUTININ INDUCED HEMOLYTIC ANEMIA IN A PATIENT WITH

Lohmror Anurag , Choudhary Richa

Received: 30 June 2015

Autoimmune hemolytic anemias (AIHA) are an uncommon group of disorders

blood count and platelet count. The peripheral blood smear

Int J Med Res Health Sci. 2015;4(4):911-912

based on the ability of the autoantibodies to attach to and

Friedberg R C. Autoimmune Haemolytic Anaemias. In:

Int J Med Res Health Sci. 2015;4(4):911-912

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