13. The Napkin
That day we were on call together again, me and Peter. And I don’t know why, but the hospital was calmer than ever, we hadn’t seen a single case the whole night. We had already played some cards, but Peter sucked at it and I was tired of winning.
He was throwing a ball at the wall and playing catch with himself, and I was reading a book I can’t remember the name of. It was remarkable how the hours passed so slowly when we were doing nothing (and so fast when there were a million patients to see).
‘Are you sure there’s no one else out there? Not a single soul in Brooklyn needed to come to the hospital today? Come on, I can’t believe it.’ Peter said frustrated.
‘Unfortunately, I don’t think anything is different from 5 minutes ago when you asked the first time.’ We laughed. ‘But you know we shouldn’t talk like that in a shift right. That’s the keyword to make everything go South.’ He passed his fingers through his lips zipping his mouth.
A while later I decided to give up on that book that wasn’t interesting anyway. Then I had an idea, I asked him to tell me some interesting case I didn’t follow up on so we could have some fun.
‘That’s actually not a bad idea, Diaz.’ He threw the ball at me. ‘In fact, I have a case we don’t know what it’s about yet. Maybe your brain can help me figure this out.’
‘Let’s see.’
‘A while ago I saw this girl right after she was admitted. 21 years old or something like that. She was complaining of a persistent headache that according to her it was the worse of her life. Besides that, she was having abdominal pain and fever for 1 month then.’
‘Hum... okay, you got my attention.’
He smiled. He loved to tell stories, you could see how excited he was.
‘So, we decided to order an MRI because of the severity of the pain, and it showed multiple hemorrhages across the brain along with venous thrombosis. We also ordered a blood test that showed anemia and thrombocytopenia.’
‘Wait, we should write that down.’ I got up and took a whiteboard marker from my purse.
‘Do you carry that around with you?’ He laughed. ‘Are you a teacher or something?’
‘In fact, I do, and it always comes in handy. You’d be surprised.’
However, the whiteboard wasn’t in that room, so I had to settle for a napkin I found inside my purse.
I started writing on it: ‘Thrombosis, fever, abdominal pain, bicytopenia.’
‘Go on, Peter.’
‘Okay. So, because of the bicytopenia and thrombosis, we started thinking autoimmune. We ordered all the antibodies but they were all negative.’
‘Any other important lab results?’
‘Yes, she had a raised LDH, slightly elevated bilirubin, and negative direct Coombs test. We also performed a bone marrow biopsy which was hypercellular but without other abnormalities.’
‘Hum, so she had hemolysis, right? I bet you missed the fact that she was jaundiced on your physical.’ I teased him.
‘Very funny! And no I didn’t. I just forgot to mention it to you.’ He shrugged. ‘But yes, she had hemolysis.’ I put it on the napkin.
‘Intra or extravascular?’
‘We are not sure, but apparently both.’
‘It does look autoimmune.’ I thought about it for a moment. ’Even though her Coombs is negative.’
That was so weird. It just didn’t make sense.
‘Did you investigate her abdominal pain, Peter?’
‘Yes, and her ultrasound showed thrombosis of the hepatic vein, very suggestive of Budd Chiari. But then again, all her workup for the thrombotic states were normal. We treated the Budd Chiari but we don’t know why she had it.’
‘That’s weird.’
I scratched abdominal pain from our list.
‘Why did you scratch that?’
‘Because that was another thrombosis. It will not help us here.’ By then we had thrombosis, fever, bicytopenia, hemolysis.
‘We should go for coffee. Maybe a break will help me think better.’
‘Not that you need an excuse for coffee, Diaz.’ I rolled my eyes and we went to the cafeteria.
That day the coffee wasn’t even that bad, I have to admit. Anyway, Peter was eating a sandwich and telling me some other story I wasn’t paying attention to. My mind kept going back to the case.
‘I just can’t stop thinking of autoimmune.’
‘You were not listening to me, were you?’ He seemed frustrated. ‘Okay, back to the case.’
‘Sorry, Peter, I just can’t turn it off.’ I made an apologetic face.
‘It’s fine.’
‘Let’s go back, I forgot the napkin there.’ I said already leaving.
‘I think her immune system must be involved somehow. It’s killing the cells, that’s why she has hemolysis and her bone marrow is hypercellular trying to compensate for it.’
‘I agree, Lisa. But why is it killing them? Based on the workup, it’s not producing any self antibodies.’
I thought about it for a moment.
‘Well, then maybe her bone marrow is producing bad cells. And that’s why her lymphocytes are killing it.’
Peter jumped from his chair.
‘That’s it, Lisa!’ He scratched fever from the napkin. ‘See?’
I kept staring at those words and dwelling on them. Thrombosis, bicytopenia, hemolysis. It looked so much like a triad I should remember. And then I did!
‘Paroxysmal Nocturnal Hemoglobinuria!’ I said excitedly.
‘Yes! Not exactly autoimmune, but her bone marrow is making cells without anchors for the inhibitors of the complement system to attach, so the immune system keeps killing them.’ He was shining with happiness. ‘Lucy is gonna love it. Thanks for the help, Lisa.’ He turned and hugged me, which felt weird. I wasn’t really that touchy. But it also felt good.
‘No problem, Allen. I was bored anyway.’ I tried to smile but I felt shy for some reason. I think he noticed but pretended not to.
‘Well, then I guess that it’s a good thing that we have a new patient.’ He pointed to Jane at the door holding a chart.
...
Want to read another case of Paroxysmal Nocturnal Hemoglobinuria?https://www.scielo.br/scielo.php?pid=S1676-24442016000500307&script=sci_arttext
Want to read more about Paroxysmal Nocturnal Hemoglobinuria?
https://rarediseases.org/rare-diseases/paroxysmal-nocturnal-hemoglobinuria/
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