P45 Macrophage activation syndrome: experience from a tertiary referral centre

Pinto, Lígia A.; Kagalwala, F; Singh, Suvir; Balakrishnan, C; Prabhu, SwetaR; Khodaiji, Shanaz

doi:10.1016/s0973-3698(10)60274-9

Cited by 4 publications

(5 citation statements)

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“…We also kept macrophage activation syndrome (MAS) as our differential, which is a common lifethreatening complication in several autoimmune diseases including SLE. 20 The laboratory profile of MAS should include cytopenia with sudden drop of ESR in complete blood count, hypertriglyceridaemia and raised serum ferritin (>5000-10,000 ng/mL). 21,22 Though our patient had high serum ferritin level (>40,000 ng/mL), presence of high ESR and normal triglyceride exclude macrophage activation syndrome in our patient.…”

Section: Discussionmentioning

confidence: 99%

A 16-Year-Old Girl with Systemic Lupus Erythematosus (SLE) with Septicaemia as Initial Presentation

2020

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Sepsis is a life-threatening condition caused by systemic host response to infection. Sepsis or septic shock is considered to be rare as initial presentation of autoimmune disease like systemic lupus erythematosus (SLE). SLE is a multisystem disorder which usually occurs in adolescent girls. So when an adolescent girl presents with septicaemia and multi-organ dysfunction, an autoimmune disease like SLE should be excluded. Here we report a 16-yearold girl initially presenting with septic shock along with multiorgan dysfunction. The patient was thoroughly evaluated and shock was managed effectively according to protocol. Later on she was diagnosed as a case of systemic lupus erythematosus (SLE). J Enam Med Col 2020; 10(1): 49-54

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Section: Discussionmentioning

confidence: 99%

A 16-Year-Old Girl with Systemic Lupus Erythematosus (SLE) with Septicaemia as Initial Presentation

2020

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“…Although the demonstration of hemophagocytosis in the bone marrow of lymph node is virtually diagnostic, negative results may be reported due to sampling difficulties or timing of the procedure. [ 10 ]…”

Section: Discussionmentioning

confidence: 99%

Cytology of secondary hemophagocytic lymphohistiocytosis masquerading as lymphoma in a nonimmunocompromised adult

Rekha

Kiran

Nandini

et al. 2014

J Cytol

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Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal immune disorder which is uncommon in a nonimmunocompromised adult. A 27-year-old female who presented with fever, hematuria, generalized lymphadenopathy was clinically suspected to have lymphoma was subjected to fine needle aspiration of cervical lymph nodes. Cytology of lymph node had numerous histiocytes with phagocytosed lymphocytes, red blood cells and nuclear debris. A diagnosis of secondary HLH was made based on cytological findings, clinical manifestations, and laboratory results. She was treated with steroids and recovered completely. A high degree of clinical suspicion coupled with comprehensive cytology with fine needle aspiration cytology is fruitful in the diagnosis of HLH, a potentially fatal disease and help in the delineation of therapeutic regimen.

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“…The central pathophysiology in MAS is cytokine dysfunction, resulting in an uncontrolled accumulation of activated T lymphocytes and histocytes in many organs and active hemophagocytosis by normal‐appering macrophages. There is impaired cytotoxic function of NK cells and CD8+ T cells, leading to persistent of infecting organism, causing persistent T‐cell stimulation …”

Section: Discussionmentioning

confidence: 99%

“…Clinical features of MAS overlaps with familial hemophagocytic syndromes, rare diseases with dramatic prognosis, necessitating immune chemotherapy and in many cases bone marrow transplant. 7,8 The central pathophysiology in MAS is cytokine dysfunction, 9,10 resulting in an uncontrolled accumulation of activated T lymphocytes and histocytes in many organs and active hemophagocytosis by normalappering macrophages. There is impaired cytotoxic function of NK cells and CD8þ T cells, leading to persistent of infecting organism, causing persistent T-cell stimulation.…”

Section: Discussionmentioning

confidence: 99%

Macrophage activation syndrome induced by A/H1N1 influenza in cystic fibrosis

et al. 2013

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Bacterial respiratory infections have an important impact on the development and progression of pulmonary disease in cystic fibrosis (CF). Viral infections are possible triggers of acute deterioration in the clinical status of CF patients. Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic disease characterized by pancytopenia, hepatitis, hyperferritinemia, coagulopathy, and neurologic symptoms. This syndrome is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and well-differentiated macrophages, leading to widespread hemophagocytosis and cytokine overproduction. Here, we report the case of a boy affected by CF who developed MAS triggered by pandemic H1N1 influenza; good clinical response was obtained through high dose prednisone treatment.

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P45 Macrophage activation syndrome: experience from a tertiary referral centre

Cited by 4 publications

References 0 publications

A 16-Year-Old Girl with Systemic Lupus Erythematosus (SLE) with Septicaemia as Initial Presentation

A 16-Year-Old Girl with Systemic Lupus Erythematosus (SLE) with Septicaemia as Initial Presentation

Cytology of secondary hemophagocytic lymphohistiocytosis masquerading as lymphoma in a nonimmunocompromised adult

Macrophage activation syndrome induced by A/H1N1 influenza in cystic fibrosis

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