Rare Case of Isolated Plasmodium Vivax Malaria Presenting with Pancytopenia: A Case Report
Background
Plasmodium vivax is one of the most widely distributed specie of genus plasmodium causing infection in humans with approximately 80 million new cases annually. Although severe infection with P. vivax is extremely rare Kochar et al has reported a case of P. vivax presenting with severe infection leading to severe anemia, renal involvement and ARDS with multiorgan failure. Pancytopenia is an extremely rare complication of P. vivax malaria with various proposed mechanisms including macroangiopathic hemolytic anemia, hemophagocytic syndrome and direct bone marrow suppression [1]. This is one such case of isolated vivax malaria presenting with pancytopenia
Case Presentation
A 17-year-old girl with no previous co-morbids presented with high grade fever associated with rigors and chills, vomiting, loose motions and decrease appetite for 5 days. On examination patient had Bp 100/70, Pulse 92/min, Temperature 101F, Oxygen saturation 98% with room air. She was dehydrated and pale with bruises on arms and thighs. There was no jaundice or lymph nodes. On abdominal examination Abdomen was soft non-tender with palpable spleen with a span of about 2 fingers breath below the costal margin. Liver was not palpable. Cardiovascular and Respiratory examination was unremarkable. Complete blood picture showed TLC 2740/microliter, Hemoglobin 10.4 g/dl and Platelets 16000/microliter. Liver function tests, Renal function tests, Serum electrolytes, Urine routine examination and Erythrocyte sedimentation rate were normal. C-reactive protein was 48, LDH 443, Ferritin 1021 and D-dimers were 890. Peripheral film showed Retic count of 3%. Dengue serology and Covid-19 PCR was negative. Blood and Urine Cultures showed no growth. Ultrasound abdomen showed splenomegaly with size of 13 cm. Peripheral smear for Malarial Parasite showed early trophozoites of Plasmodium vivax. Patient was started on Intravenous Artesunate as she could not tolerate Oral anti-malarial. Her Hematologic parameters dropped further during her stay at the hospital with TLC to 2520/ microliter, Hb to 8.5 g/dl and platelets to 11000/ microliter. Patient responded well to Intravenous artesunate. She became afebrile and her blood parameters normalized after 5 days. She was discharged with follow up test for G6PD assay after one week.
Discussion
Malaria is a worldwide national health problem in many
countries, and many occur in tropical and subtropical areas
including sub-Saharan Africa, Asia and Latin America [2].
Worldwide approximately 3 billion people resides in areas which
have high risk of malaria transmission. 1.1 to 2.7 million deaths
occur due to severe malaria every year. Malaria is considered as a
5th leading cause of death due to infectious disease and 2nd leading
causes of death in Africa with 1 million people dying every year in
Africa. About 85% of all the malarial cases in the world are due to
Plasmodium falciparum with Plasmodium vivax on 2nd number. 90%
of all the malarial deaths occur in Africa [3].
Of all the plasmodium species, the deadliest and the most virulent
is P. falciparum which can lead to life threating complications and
death if not treated early. Species other than P. falciparum usually
cause mild disease with minimal complications [2]. P. vivax malaria
is a parasitic infection which is carried by Anopheles mosquito [4].
Life cycle of P. vivax is a unique phase known as hypnozoite stage in
which the parasite remains dormant in the liver causing frequent
relapses after acute infection is treated. Severe complications due
to P. vivax are extremely rare compared to P. falciparum [5].
Hematological changes occurring in malaria include anemia,
thrombocytopenia, leucopenia, neutropenia, leukocytosis,
atypical leukocytosis and splenomegaly. One study has shown
that lymphopenia, leucopenia and thrombocytopenia are the
key predictors of malaria infection. Low Hb, High lymphocyte
count, low platelets and monocytosis are more severe in chronic
malaria compared to acute malaria [6]. Hematologic changes due
to malaria depend on various factors including malaria endemicity,
background hemoglobin disorders, demographic factors and level
of malarial immunity. Thrombocytopenia and anemia are the main
blood abnormalities occurring in malaria [3]. Pancytopenia is an
uncommon manifestation of malaria and is mostly common with
P. falciparum and occur due to both direct and indirect effect of
infection on hematopoietic cells in the bone marrow [7]. Two main
mechanisms involved in pancytopenia involve direct bone marrow
suppression and hemophagocytosis, the latter is mostly reported
with P. falciparum malaria.
P. vivax has lesser pyrogenic threshold, marked inflammatory
response and very high cytokine production as compared to P.
falciparum. There have been case reports of severe disseminated
infection with multi organ failure due to P. vivax. Studies have also
shown that P. vivax can lead to acute tubular necrosis and acute
interstitial nephritis termed as Malarial Nephropathy [1].
Pancytopenia secondary to P. vivax malaria is extremely rare
and has only been reported in 0.9% of the confirmed P. vivax cases.
To the best of author’s knowledge, only up to 5 cases of isolated P.
vivax malaria with pancytopenia have been reported in literature
without other associated comorbids [2,5]. Key mechanism involved
in the development of pancytopenia in P. vivax is microangiopathic
hemolytic anemia followed by hemophagocytic syndrome which has
rarely been reported with P. vivax [5]. Hemophagocytic syndrome is
an unusual clinicopathological syndrome that is characterized by
overt immunological responses by T-cells producing high levels
of interferon gamma, TNF alpha, IL-1, IL-2 and IL-18 leading to
activation of macrophages which in turn causes phagocytosis of
hematopoietic cells and bone marrow suppression. It can be fatal if
misdiagnosed or diagnosis is delayed.
Hemophagocytic syndrome mainly occurs secondary to many
infections including viral, bacterial, fungal and parasitic infections
[4]. Estimation of exact prevalence of malaria associated HLH is very
difficult because bone marrow biopsy is not routinely performed
to diagnose malarial infection and exact mortality rate is also not
low. It can be fatal if not treated. Malaria associated phagocytic
syndrome can lead to prolong hemophagocytosis which is a very
rare complication and can lead to prolong anemia. It has been
reported only with P. falciparum and not with P. vivax [2]. Various
treatment modalities to treat HLH include treating the causative
agent, immunosuppressant, steroids and IV immunoglobulins [8].
Various studies have proven that malaria associated HLH has an
excellent response to antimalarials and supportive care without
any need for immunosuppressants and steroids [2,8].
Malaria must always be kept in differential diagnosis as a
cause of prolong fever with refractory anemia and pancytopenia
particularly in endemic areas and asymptomatic patient despite of
negative smear and rapid antigen test. Bone marrow biopsy is the
key to diagnose P. falciparum malaria in such cases [9,10].
Conclusion
Malaria is one of many infections which can involve any organ of the body especially the bone marrow. Bone marrow involvement causes decrease in all the three cell lines leading to pancytopenia. It is important that malaria should always be included in the differential diagnosis whenever pancytopenia is worked up because it is one of the treatable causes of pancytopenia with excellent prognosis.
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