Hemophagocytic lymphohistiocytosis in leprosy

Høyvoll, Liv R.; Fløisand, Yngvar; Orrem, Hilde Lang; Gunnarsson, Ragnar; Landrø, Linn; Brevig, Trine; Gaustad, Peter; Nordøy, Ingvild

doi:10.47276/lr.86.4.403

Cited by 4 publications

(2 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[ 2 ] The persistence and proliferation of lepra antigens can cause uncontrolled cytokine storm and activation of macrophages leading to a defective T-cell and NK-cell function, thus causing HLH. [ 3 4 5 ] Despite similar pathophysiology, the prevalence of HLH in ENL is very rare, and to the best of our knowledge, there are only three case reports to date in this regard. [ 1 3 4 ] We present here a case of a 30-year-old female with ENL who progressed to HLH.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Hemophagocytic lymphohistiocytosis in erythema nodosum leprosum: Case report of an unusual conundrum

Mishra

Krishnan

Madakshira

et al. 2022

Indian Dermatol Online J

View full text Add to dashboard Cite

Hemophagocytic lymphohistiocytosis (HLH) and erythema nodosum leprosum (ENL) result from a complex agent–host interaction and form a continuum of the same spectrum. A 30-year-old multi-gravida presented at 36 weeks gestation with fever and erythematous raised lesions over the face and upper and lower limbs after defaulting treatment for borderline lepromatous leprosy. Skin biopsy confirmed ENL, hence multi-drug therapy (MDT) and oral steroids were restarted. However, her condition worsened and she developed icterus, periorbital puffiness, pleural effusion, ascites and splenomegaly. Laboratory investigations showed pancytopenia, conjugated hyperbilirubinemia, transaminitis, elevated lactate dehydrogenase, hypertriglyceridemia, hyperferritinemia and hypofibrinogenemia. Dapsone was stopped on the suspicion of dapsone hypersensitivity but hyperbilirubinemia progressed. Diagnosis of HLH was clinched after bone marrow aspirate showed florid hemophagocytosis and subsequently, intravenous immunoglobulin (2 g/kg) over 5 days and dexamethasone were administered. The patient improved gradually with normalization of laboratory parameters and restarted MDT. This case depicts a rare and potentially catastrophic complication of ENL and emphasizes a vigil for HLH syndrome in such cases.

show abstract

Section: Introductionmentioning

confidence: 99%

“…[ 3 4 5 ] Despite similar pathophysiology, the prevalence of HLH in ENL is very rare, and to the best of our knowledge, there are only three case reports to date in this regard. [ 1 3 4 ] We present here a case of a 30-year-old female with ENL who progressed to HLH. Informed consent of the patient was duly taken.…”

Section: Introductionmentioning

confidence: 99%

Hemophagocytic lymphohistiocytosis in erythema nodosum leprosum: Case report of an unusual conundrum

Mishra

Krishnan

Madakshira

et al. 2022

Indian Dermatol Online J

View full text Add to dashboard Cite

show abstract

A life-threatening consequence of erythema nodosum leprosum

Malhi,

Singla,

Singh

et al. 2024

IJDVL

View full text Add to dashboard Cite

Borderline lepromatous leprosy: A case report

Fernando,

Welhenge,

Premaratna

et al. 2024

Asian Pacific Journal of Tropical Medicine

View full text Add to dashboard Cite

Rationale: Lepromatous leprosy can have many atypical presentations, obscuring early diagnosis. We present a case of lepromatous leprosy, presenting with atypical features, which made a diagnostic dilemma. Patient concerns: A 48-year-old man presented with bilateral lower limb oedema and scaly “ichthyosis like” skin rash in both hands and feet, hepatosplenomegaly and pancytopenia, over a course of three months, without any classical features of leprosy. A skin biopsy revealed an unexpected diagnosis of borderline lepromatous leprosy. Diagnosis: Lepromatous leprosy. Interventions: Multi-drug regimen treatment with rifampicin, dapsone and clofazimine for lepromatous leprosy. Outcomes: The patient made a good clinical recovery. Lessons: In endemic settings, clinicians should be aware of similar atypical manifestations of leprosy to face the global challenge of eradicating this chronic deforming disease.

show abstract

Hemophagocytic lymphohistiocytosis in leprosy

Cited by 4 publications

References 0 publications

Hemophagocytic lymphohistiocytosis in erythema nodosum leprosum: Case report of an unusual conundrum

Hemophagocytic lymphohistiocytosis in erythema nodosum leprosum: Case report of an unusual conundrum

A life-threatening consequence of erythema nodosum leprosum

Borderline lepromatous leprosy: A case report

Contact Info

Product

Resources

About