ABSTRACT
Introduction and importance: Hemophagocytic lymphohistiocytosis
(HLH) is a fatal disease caused by the overactivation of tissue
macrophages in response to environmental factors. Because HLH has many
clinical symptoms with malaria, which is the underlying cause,
identifying the condition can be difficult.Case presentation: We present a rare case of HLH triggered by
malaria in a 15-year-old male patient of paroxysmal cold hemoglobinuria
(PCH), which developed as a response to latent syphilis. The patient
presented with fever and hematuria, diagnosed with malaria by Plasmodium
Falciparum; he was started on Artesunate with anemia correction and cold
avoidance. After antimalarial therapy, the patient’s clinical condition
worsened, and he met the criteria for HLH. Treated with IV dexamethasone
and injection epokine under strict surveillance the patient showed
complete recovery.Clinical Discussion: Pakistan has reported an only case of
malaria with Plasmodium vivax and HLH. Nonetheless, several instances of
Plasmodium falciparum and vivax infections have been documented in China
and India. Patients in each of these situations have a decent prognosis.
The fatality rate from HLH is not widely known, however secondary HLH is
fatal without treatment.Conclusion: This report aims to alert physicians, about the
complication of HLH in malaria and to present a model approach to
diagnose and treat it when linked to
PCH.Keywords: Paroxysmal cold hemoglobinuria,
hemophagocytic lymphohistiocytosis, malaria, case report
1 INTRODUCTION Hemophagocytic
lymphohistiocytosis (HLH) is a sporadic syndrome of pathologic immune
activation caused by a variety of environmental triggers such as
infection, cancer, and rheumatologic disorders. Because of the HLH
syndrome’s rarity, diversity, and complexity, diagnosis is difficult
[1]. Malaria, a vector-borne disease caused by Plasmodium species is
a rare cause of HLH, hence it is difficult to diagnose due to
significant overlap in clinical features and laboratory findings between
these two entities, which can be complicated by pre-existing paroxysmal
cold hemoglobinuria (PCH) rarely found in the literature [2]. PCH is
a type of autoimmune hemolytic anemia (AIHA) caused by atypical IgG
autoantibodies that bind to their target RBC antigen and fix complement
at 4 °C. After complement activation, cold-reactive antibodies primarily
cause intravascular hemolysis producing symptoms including
hemoglobinuria, pallor, jaundice after cold exposure [3]. We present
the first reported case of HLH in a 15-year-old patient with PCH
triggered by acute febrile illness secondary to malaria. This case
underscores the importance of considering a broad differential diagnosis
in patients presenting with fever and hemolysis, particularly in areas
with a high prevalence of malaria. Additionally, it highlights the need
for prompt recognition and management of HLH, as it can have
life-threatening complications if left untreated.