International Journal of Contemporary Pediatrics

Reddy YRKK et al. Int J Contemp Pediatr. 2023 Oct;10(10):1587-1589

http://www.ijpediatrics.com

pISSN 2349-3283 | eISSN 2349-3291

Case Report

DOI: https://dx.doi.org/10.18203/2349-3291.ijcp20232889

Mefenamic acid triggered autoimmune hemolytic anemia in a child: a rare case report

Y. Ravi Kiran Kumar Reddy, Saiprasad Onkareshwar Kavthekar, Narahari Venkata Susmitha*, Nivedita Balasaheb Patil, Priti Kamble, Chilukuru J. Satyamani Deepika

Department of Paediatrics, Dr. D. Y. Patil Medical College, Hospital and Research Institute, D. Y. Patil Education Society (Deemed to be University), Kolhapur, Maharashtra, India

Received: 26 July 2023

Accepted: 02 September 2023

*Correspondence:

Dr. Narahari Venkata Susmitha,

E-mail: susmitha.narahari@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

Autoimmune hemolytic anemia (AIHA) is characterized by the abnormal production of antibodies that bind to antigens on the surface of red blood cells (RBC), resulting in RBC destruction. Here we describe a rare case of mefenamic acid-induced AIHA in a 3-year-old boy who developed acute hemolysis after ingestion of mefenamic acid for febrile illness. The aim is to draw the attention of pediatricians to one of the side effects of mefenamic acid, the drug which is very commonly used as an antipyretic.

Keywords: Adverse reaction, AIHA, Children, Mefenamic acid

INTRODUCTION

Autoimmune hemolytic anemia (AIHA) is a rare disease characterized by reduced RBC survival and the presence of autoantibodies to autologous RBCs. There is hemolysis with occasional pancytopenia along with positive direct Coomb’s test (DCT). Drug-induced AIHA was first described by Snapper (1953) after the occurrence of DCT-positive hemolysis and pancytopenia following ingestion of mephenytoin.1 Based on the stimulation of antibody production, AIHA is further classified into hot and cold AIHA or mixed AIHA. The incidence in children is 1 in 100,000 and the most common age range is 2-12 years.2-4

We describe a rare case of mefenamic acid-triggered AIHA in a 3-year-old boy who developed acute hemolysis after taking mefenamic acid for fever.

CASE REPORT

A 3 years boy presented to us with complaints of acute onset of dark brown colored urine and severe pallor. There was history of taking mefenamic acid suspension three doses (Total 300 mg) for the treatment of fever. The fever was for 2 days which was acute in onset and intermittent probably a viral fever. There was no history suggestive of any hematological or immunological illness in the past. On examination patient was irritable with severe pallor and lemon-yellow icterus (Figure 1), persistent tachycardia with spleeno-hepatomegaly and signs of impending cardiac failure. Complete blood count showed Hemoglobin of 2.9 gm/dl, Total WBC counts of 11,540/cu mm and platelets of 4.44 lakh/uL with corrected retic count 0.62%. The peripheral smear showed microcytic hypochromic anemia with autoagglutination. (Figure 2 and 3). The liver function test was abnormal (Total bilirubin: 3.10 mg / dl and Indirect bilirubin 2.70 mg/dl). The DCT was positive and urine analysis had shown hemolysis. The renal function test was sightly deranged.

The patient was diagnosed as a case of drug induced coomb’s positive AIHA triggered by mefenamic acid ingestion based on clinico-hematological parameters. He was treated with injection methylprednisolone @ 30 mg/kg for 3 days. He also received PCV transfusion 5 ml/kg twice without any significant adverse reactions. On day three, rise in the hemoglobin without any signs of hemolysis was noted and he was symptomatically and hemodynamically better. From day 4, patient was put on tab prednisolone at the dose of 2 mg/kg/day for five days and was discharged with advice to continue oral steroid for total duration of 4 weeks in tapering doses.

Figure 1: Pallor and icterus in our patient.
Figure 1: Pallor and icterus in our patient.
Figure 2: Complete blood count report of our patient.
Figure 2: Complete blood count report of our patient.
Figure 3: Peripheral smear with autoagglutination.
Figure 3: Peripheral smear with autoagglutination.

DISCUSSION

AIHA is a rare condition and there is production of antibodies against the surface RBC antigens. It is predominantly an acute onset disease lasting for 3-6 months in 70-80% of cases. Chronic hemolysis develops in a quarter of patients with an underlying secondary cause.5 The appearance of jaundice and abnormally dark colored urine along with anemia is suggestive of hemolysis, but it presents only in 60% of patients. A positive DCT which demonstrates the presence of antibodies or complement on the surface of RBCs, an elevated reticulocyte count in the background of hemolysis are the cornerstones in the diagnosis of AIHA.6

AIHA induced by drugs is on the rise, predominantly due to nonsteroidal anti-inflammatory drugs (NSAIDS) such as ibuprofen, naproxen, mefenamic acid and aspirin. Garratty et al updated the drug list and reported 125 drugs responsible for AIHA.7 There are three mechanisms of drug-induced AIHA either autoimmune, drug adsorption or immune complex.8 Mefenamic acid was the most common cause of drug induced AIHA among NSAIDs, which was caused by an autoimmune mechanism. Similar to our case report, Mishra et al reported a case of mefenamic acid induced AIHA.9

In our case, DCT was positive indicating the presence of autoantibodies against RBCs while presence RBCs in urine and raised unconjugated bilirubin indicated hemolysis. Although, all the clinical features and investigations pointed towards AIHA, it is always better to first rule out other causes of hemolysis like microangiopathy, hereditary conditions (i.e., Sickle cell anemia, G6PD deficiency and Hereditary spherocytosis) and malaria. As there was no previous history of drug exposure and DCT was positive, we were able to rule out G6PD deficiency. Similarly other causes of hemolysis were ruled out.

The drug of choice for the treatment in AIHA is systemic steroids. The mechanism of action of steroids is probably down-regulation of Fc receptors on phagocytes and reduced IL-2 production.10 The packed RBC transfusion is rarely required in AIHA but in our case it was given as patient’s hemoglobin was under 3gm/dl and there was persistent tachycardia, breathlessness, and signs of impending cardiac failure. Intravenous immunoglobulin, rituximab and plasmapheresis are other modes of treatment in AIHA.

CONCLUSION

Mefenamic acid can trigger drug induced AIHA in children and the treating pediatrician should be aware of this adverse reaction of mefenamic acid which is commonly prescribed as an antipyretic in children.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

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