Immunology Question of the Day | Clinical Case Discussion by Dr. Mukesh Bhatia

[00:00:00]Hello friends.

[00:00:01]Today I'll be discussing a question of immunology with you.

[00:00:06]So here's the question.

[00:00:09]And see the answers.

[00:00:12]And do the write down the answer in your copy. A 12-year-old girl with a systemic juvenile idiopathic arthritis develops persistent high-grade fever, hepatosplenomegaly, altered sensorium and mucosal bleeding.

[00:00:29]Lab evaluation shows marked hyperferritinemia.

[00:00:34]That means serum ferritin level is more than 15,000 ng/mL.

[00:00:40]Pancytopenia, elevated liver enzymes, high triglyceride and low fibrinogen.

[00:00:48]Which of the following is the most likely diagnosis?

[00:00:52]The options are macrophage activation syndrome, disseminated intravascular coagulation, septic shock and autoimmune hepatitis.

[00:01:02]Well, I give you some time to think the answer.

[00:01:07]Read it carefully.

[00:01:09]Write down the answer in your copy.

[00:01:11]Although I'm going to the next slide to give you answer, but if you need more time, you can stop the video to see it.

[00:01:18]So I hope by now you have written the answer.

[00:01:22]Answer is macrophage activated syndrome.

[00:01:25]Let's discuss this question in detail.

[00:01:29]So MAS is a life-threatening hyperinflammatory syndrome caused by uncontrolled activation of macrophages and cytotoxic T cell.

[00:01:41]Most commonly complicate systemic juvenile idiopathic arthritis and adult-onset Still's disease.

[00:01:50]Hallmark laboratory finding include very high serum ferritin like in this case, pancytopenia, high triglyceride, hypofibrinogenemia and elevated transaminases.

[00:02:02]All these were there in our patient.

[00:02:05]Bone marrow may demonstrate hemophagocytic cytosis, although its absence does not rule out MAS.

[00:02:15]This is considered as a secondary form of hemophagocytic lymphohistiocytosis.

[00:02:21]Initial treatment consists of high doses of corticosteroid with anakinra, which is an interleukin-1 inhibitor.

[00:02:29]And in anakinra, we primarily use in refractory cases.

[00:02:37]Early recognition is essential because uh delayed treatment carries very high mortality.

[00:02:44]Always suspect MAS in a patient with sJIA who suddenly deteriorate and with extreme hyper- ferritinemia.

[00:02:55]So, last-minute revision point about macrophage activation syndrome.

[00:03:01]Suspect if seen in patient of sJIA.

[00:03:04]Ferritin level is in thousands of nanogram per ml.

[00:03:08]Pancytopenia, as you think about MAS unless proven otherwise, such a diagnostic feature.

[00:03:16]Thank you very much.