International Journal of Research in Medical Sciences

Deepak S et al. Int J Res Med Sci. 2023 Sep;11(9):3332-3335

www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012

DOI: https://dx.doi.org/10.18203/2320-6012.ijrms20232788
Original Research Article

Study of hematological profile of systemic lupus erythematosus

Shipra Gulati1, Vikas Kumar2, Preshika Rawat2*, Kunal Chawla1,
Rishikesh Dessai1, Sunil Jain1

1
Department of Medicine, 2Department of Internal Medicine, Sir Ganga Ram Hospital, Old Rajinder Nagar, New
Delhi, India

Received: 06 July 2023

Revised: 03 August 2023
Accepted: 14 August 2023

*Correspondence:
Dr. Preshika Rawat,
E-mail: preshikarawat999@gmail.com

Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under
the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial
use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Background: Hematological abnormalities are prevalent in systemic lupus erythematosus (SLE), with approximately
72% of patients experiencing anemia, primarily in the form of autoimmune hemolytic anemia. Other manifestations
include leukopenia (32%), lymphopenia (54%), and thrombocytopenia (23%). This study aimed to further investigate
these hematological manifestations, which may serve as presentations of SLE and might be overlooked if suspicion
levels are low.
Methods: A descriptive observational study was conducted over 18 months at a Sir Ganga Ram hospital, a tertiary
care centre. One hundred thirteen SLE cases, comprising newly diagnosed patients and previously diagnosed patients’
records, were reviewed, with 13 cases excluded based on exclusion criteria. One hundred patients with hematological
abnormalities and fulfilling ≥4 SLICC criteria for SLE diagnosis were included in the study.
Results: One hundred cases of SLE with hematological abnormalities (88 women, 12 men) were analyzed. At
presentation, 83% (n=84) of patients displayed hematological manifestations. The most prevalent abnormality was
anemia, present in 72% of the study group, with a mean hemoglobin level of 10.073 gm/dl. Additionally, leukopenia,
lymphopenia, thrombocytopenia, and pancytopenia were observed in 32%, 54%, 23%, and 14% of cases,
respectively. Neutropenia was detected in only 5% of cases.
Conclusions: Hematological manifestations are the most common presenting signs of SLE in North India. Anemia,
with a multifactorial basis, is the most frequent hematological abnormality throughout the disease course. A high
index of suspicion is crucial when evaluating cases of anemia in daily clinical practice.

Keywords: Anemia of chronic disease, Autoimmune hemolytic anemia, Iron deficiency anemia, Leukopenia,
Systemic lupus erythematosus, Thrombocytopenia

INTRODUCTION investigation into the diagnosis of SLE.2,3 Patients with

SLE may initially present with constitutional,
Systemic lupus erythematosus (SLE) is a chronic musculoskeletal, dermatologic, renal, neuropsychiatric,
autoimmune inflammatory disease that has protean pulmonary, gastrointestinal, cardiac or hematological
manifestations and follows a relapsing and remitting features.4
course. Over 80 percent of cases occur in women during
their childbearing age.1 SLE can affect almost any organ Patients may present with any of the following
system; thus, its presentation and course are highly manifestations: constitutional (e.g., fatigue, fever,
variable, ranging from indolent to fulminant. The arthralgia, weight change; musculoskeletal (e.g.,
classical presentation of a triad of fever, joint pain and arthralgia, arthropathy, myalgia, frank arthritis, avascular
rash in a woman of childbearing age should prompt necrosis); dermatologic (e.g., malar rash,

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 Deepak S et al. Int J Res Med Sci. 2023 Sep;11(9):3332-3335

photosensitivity, discoid lupus); renal (e.g., acute or induced bone marrow suppression, infection, etc.)
chronic renal failure, acute nephritic disease); causes.12,13
neuropsychiatric (e.g., seizure, psychosis); pulmonary
(e.g., pleurisy, pleural effusion, pneumonitis, pulmonary Objective
hypertension, interstitial lung disease); gastrointestinal
(e.g., nausea, dyspepsia, abdominal pain); cardiac (e.g., The objective of this study was to investigate further, the
pericarditis, myocarditis); hematologic (e.g., cytopenias hematological manifestations which may be the
such as leukopenia, lymphopenia, anemia, or presentation of SLE and may be missed if the index of
thrombocytopenia).4 suspicion is low.

Hematologic manifestations in SLE METHODS

All the cellular elements of the blood and coagulation The study was conducted in the department of internal
pathway can be affected in SLE patients. SLE is medicine at Sir Ganga Ram Hospital in New Delhi, India,
immunological disease, so hematological manifestations from December 2015-July 2017, following approval from
are expected to be more common than the other features, the hospital’s Ethics Committee. It was a descriptive
since antigens are more often present in blood and blood study including 100 patients, who were either newly
vessels than any other organ and in SLE auto antibodies diagnosed case of SLE (using SLICC criteria) or
are developed against any antigens and tissues.5 previously diagnosed cases obtained from the hospital
records. Patients under 18 years of age, pregnant women,
Sometimes, hematological abnormalities can be caused patients who were on treatment were excluded from the
by the pathophysiology of SLE itself, but times they can study.
be present in SLE due to some other etiology found in
patients with SLE but not be a manifestation of SLE. It is Hematological profile of both the group of patients were
important to distinguish hematological abnormalities as obtained and recorded. Descriptive statistics were
either manifestations of SLE, consequence of SLE analysed with SPSS version 17.0 software. Continuous
treatment or as part of another blood cell dyscrasias. variables were presented as mean±SD. Categorical
variables were expressed as frequencies and percentages.
Anemia is a common hematological abnormality in SLE. Association between two or more variables were
The most frequent cause of anemia in SLE is suppressed compared using chi squared test. P<0.05 was considered
erythropoiesis from chronic inflammation (anemia of statistically significant.
chronic disease) which is the most common form (60-
80%).6 Other causes are iron deficiency anemia, RESULTS
autoimmune haemolytic anemia and anemia due to blood
loss. Out of 100 patients that were found to have SLE during
the study period 88% were females and 12% were males
Leukopenia in SLE may occur as a result of thus clearly indicating preponderance for females (Table
lymphopenia, neutropenia or a combination of both. The 1).
prevalence of lymphopenia in SLE ranges from 20 to
81% and its degree may correlate with disease activity. Table 1: Gender distribution.
Both T and B lymphocytes are reduced, while natural
killer cells are typically increased.7 Leukopenia patients Sex Frequency Percentage Cumulative
with SLE are found to have reduced surface expression of Female 88 88.0 88.0
complement regulatory proteins CD55 and CD59.8,9 Male 12 12.0 100.0
Deficiency of these proteins may make these cells Total 100 100.0
susceptible to complement-mediated lysis. There is
increasing evidence that endogenous production of type 1
Table 2: Age distribution.
interferons (IFNs) is implicated in the pathogenesis of
neutropenia and lymphopenia in SLE. Elevated serum
Age Frequency % Cumulative %
levels of IFN-a in SLE correlate inversely with leucocyte
≤20 years 3 3.0 3.0
numbers.10,11
21-30 years 26 26.0 29.0
Thrombocytopenia with platelet count of <100000/mm3 31-40 years 26 26.0 55.0
may appear in 10-25% of SLE patients. It has a reported 41-50 years 21 21.0 76.0
prevalence ranging from 7 to 30% in large series of 51-60 years 18 18.0 94.0
patients with SLE.11 Thrombocytopenia may, like >60 years 6 6.0 100.0
anemia, result from a number of immune (antiplatelet Total 100 100.0
antibodies, anti-thrombopoietin antibodies,
antiphospholipid antibodies, etc.) and non-immune (drug- The patient’s age at the time of presentation varied from
18 years to 60 years with a mean of 39.85 years (SD

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 Deepak S et al. Int J Res Med Sci. 2023 Sep;11(9):3332-3335

12.832 years). Out of 100 patients, 56 (56%) patients One study by Nossent et al of 126 patients with SLE
were under the age of 40 years. In male patients 9 out of showed that 47% had neutropenia, 27% had
twelve (75%) were >40 years indicating that SLE occur thrombocytopenia, 20% had lymphopenia and 13% had
in males in later part of year (Table 2). haemolytic anemia.15

Table 3: Percentage of hematological manifestation in Hematological abnormalities (anemia, leukopenia,

SLE. lymphopenia and thrombocytopenia were found to be the
most common presentation of SLE and it was the
Hematological manifestation Frequency Percentage presenting manifestation in 83% of the subjects. This
Anemia 72 72 observation is contradictory to the description of the
Leukopenia 32 32 disease in most western and Indian textbook and majority
Neutropenia 5 5 of previously conducted studies.16,17 However a
Lymphopenia 54 54 multicentre French study and a study conducted in India
Thrombocytopenia 23 23 had shown that the most common initial presentation was
hematological.5,18 The prevalence of hematological
Pancytopenia 14 14
findings in SLE in these studies were similar to that seen
in our patients.
Majority of the participants 12 (18%) and 13 (20%) had
mild to moderate anemia respectively while 2 (3%) had
The most prevalent hematological abnormality was
life threatening anemia. While, majority of the
anemia present in 72% of the patient in the study group
participants had mild leukopenia 12 (18%) while only 4
with a mean haemoglobin value of 10.073 gm/dl. This
(6%) had moderate leukopenia. Whereas, majority of the
also validates in different studies where the most
participants 11 (17%) had mild thrombocytopenia. Only 1
common presentation was anemia.15,6,19
(1.5%) of the participant had severe thrombocytopenia
(Table 3).
The most common type was AIHA which was seen in
26% of patient. This was followed by ACD and IDA
Table 4: Percentage of patients with anemia. which were seen in 10% and 9% of patients respectively.
This is in contrast to western literature according to
Anemia Frequency % Cumulative %
which the most common type of anemia was ACD.
ACD 9 9.0 9.0 According to a study done in Europe by Swaak et al,
AIHA 26 26.0 35.0 ACD was the most prevalent and seen in 27% of the
IDA 10 10.0 45.0 cases and hemolytic anemia in 5% of the cases.20 A
IDA/ACD 14 14.0 59.0 similar study done in Athens had shown that ACD was
No 26 26.0 85.0 present in 37.1%, IDA in 35.6%, AIHA in 14.4% of
Other 15 15.0 100.0 cases.21 Another study conducted in India had shown that
Total 100 100.0 AIHA was seen in 27.9% of cases.5

Of the 72% of the patients who had anemia majority were Other hematological abnormalities included were
autoimmune hemolytic anemia with 26%, IDA were leukopenia present in 32% of patients. This comprised of
10%, ACD was 9% and mixed IDA/ACD were 14%. lymphopenia in 54%, neutropenia in 5%.
(Table 4). Thrombocytopenia was seen in 23% and pancytopenia in
14% of patients. Presence of leukopenia has been
DISCUSSION associated in some studies with anti-ds-DNA positivity, a
component of the SLE diagnostic criteria and prognostic
Systemic lupus erythematosus (SLE) is a prototypic indicator.22 Similarly, our patients with cytopenia at
systemic autoimmune disease with variable multisystem diagnosis were significantly found to be positive for anti-
involvement and heterogeneous clinical features, ranging dsDNA in 34% patients. This also suggests that in
from mild to life threatening. patients presenting with leukopenia and lymphopenia,
other signs and symptoms of SLE should be carefully
In a study conducted in a tertiary care centre in North looked into and anti-dsDNA can be ordered to confirm
Kerala it was found that hematological manifestations the suspicion.
were the most common initial manifestation of SLE and
it was one of the presenting manifestations in 82% of the The observational study lacked patient follow-up,
subjects.5 hindering assessment of the persistence of hematological
abnormalities and complications. Disease activity
A series of studies reviewed by BudMan showed that correlation was not explored, and coexisting
anemia occurred in 57-78% of patients with SLE.14 ACD manifestations were disregarded. Exclusion of paediatric
was the most common type of anemia. Anemia with patients limited generalization, and the small sample size
positive coombs test was seen in 10% of the cases. may have affected statistical power.

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 Deepak S et al. Int J Res Med Sci. 2023 Sep;11(9):3332-3335

CONCLUSION 11. Hepburn AL, Narat S, Mason J. The management of

peripheral blood cytopenias in systemic lupus
Studies over a long period have demonstrated that erythematosus. Oxford Rheumatol J. 2010;49:2243-
hematological involvement is common in SLE, however 54.
in our study we concluded that hematological 12. Levine BA, Erkan D. Clinical assesment and
involvement as early as at the time of presentation is management of cytopenias in lupus patients. Curr
commonly seen in SLE patients. The most common Rheumatol Rep. 2011;13:291-9.
abnormality being anemia whose etiology is 13. Ktona E, Barbullushi M, Baka T, Idrizi A, Shpata V,
multifactorial. Thus, all cases of anemia should be Roshi E. Evaluation of thrombocytopenia in
carefully evaluated and managed. systemic lupus erythematosus and correlation with
different organs damages. Mater Sociomed.
Funding: No funding sources 2014;26:122-4.
Conflict of interest: None declared 14. Budman DR, Steinberg AD. Hematologic aspects of
Ethical approval: The study was approved by the systemic lupus erythematosus. Current concepts.
Institutional Ethics Committee Ann Intern Med. 1977;86:220-9.
15. Nossent JC, Swaak AJ. Prevalence and significance
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Journal Critique: Study of Hematological Profile of Systemic Lupus Erythematosus

The study by Gulati et al. (2023) aimed to investigate the prevalence and types of
hematological abnormalities present at the time of diagnosis in 100 patients with systemic lupus
erythematosus (SLE) at a tertiary care center in New Delhi, India. The researchers conducted a
descriptive observational study, finding that anemia was the most common hematological
manifestation (72%), followed by lymphopenia (54%), leukopenia (32%), and thrombocytopenia
(23%). Autoimmune hemolytic anemia (AIHA) was identified as the most prevalent type of anemia in
this cohort.

The strength of the study lies in its clinical relevance, addressing an important aspect of SLE,
as hematological manifestations are common and can significantly impact disease management and
outcomes, and its real-world clinical setting, enhancing the applicability of the findings to routine
clinical practice. The study specifically examined hematological manifestations at the time of SLE
diagnosis, providing valuable insights into the initial presentation of the disease.

However the study is limited by its small sample size, including only 100 patients, which may
not be representative of the broader SLE population. As a descriptive study without a control group, it
lacks the ability to explore underlying mechanisms or long-term consequences of hematological
abnormalities. The single-center setting in India may limit the generalizability of the findings to other
populations and healthcare settings. The researchers did not perform statistical tests to assess the
significance of the observed differences in hematological manifestations between different subgroups
of patients. Additionally, the discussion section could have been more comprehensive, exploring the
potential implications of the findings for clinical practice and future research.

Despite these limitations, the study offers valuable insights into the high prevalence of
hematological abnormalities at the time of SLE diagnosis, particularly anemia, in an Indian cohort.
The study's emphasis on autoimmune hemolytic anemia (AIHA) as a potential cause of anemia in
SLE patients is noteworthy. Future research should address the study's limitations by conducting
larger, multi-center studies with diverse populations and comprehensive follow-up data. Such studies
would enable a more in-depth exploration of the complex relationship between hematological
manifestations and SLE, ultimately contributing to improved diagnosis, treatment, and management of
this complex autoimmune disease.

Reference:

Gulati, S., Kumar, V., Rawat, P., Chawla, K., Rishikesh Dessai, & Jain, S. K. (2023). Study of

hematological profile of systemic lupus erythematosus. International Journal of Research in

Medical Sciences, 11(9), 3332–3335. https://doi.org/10.18203/2320-6012.ijrms20232788