So we were going on 3 weeks with no diagnosis and really no new information. My fever had stopped for now, but my spleen, it kept gobbling up everything that my very robust and healthy bone marrow could make. I was better, but not really (steroids mask problems). I knew that the steroids at some point would have to stop, so they were slowly being tapered (yay sleep! Boo return of symptoms).
So let’s review at this point some symptoms: fever of unknown origin, pancytopenia (low counts) with massive splenomegaly (not just your run of the mill splenomegaly but MASSIVE- 24 cm (normal is 11 cm)), multiple lung nodules of varying caliber (lots of lung nodules of different sizes), liver lesions of undetermined size (they were less than 2 cm).
I think that my favorite doctor comment about all this was, “better to be interesting than bad.” I was interesting- yay me.
The thing is, when you are interesting, doctors talk about you. To other doctors. This is a good thing. You are getting like 20 specialists for the price of one. I heard many times, “I talked to my wife who is a stem cell doctor- she thinks this…” or “A colleague who works for Duke thinks that…”. I knew that they were intrigued and genuinely wanted to figure me out (It is like a game of Clue- I told you it was Mr. Green in the conservatory with the candlestick!). The unfortunate thing is that most doctors have only seen these rare diseases on television or in their textbooks.
We have left the realm of “common diseases occur commonly”. We may revisit it- but now we were looking for typical presentations of a rare disorder and some more common diseases with an unusual presentation. The following is a list of diseases that at one point or another were part of my differential along with their estimated prevalence in adults and the season and episode number from House MD:
Septic pulmonary embolism: unknown prevalence / season 7, episode 5
Metastatic Lung Cancer: 1 in 1,200 / season 3, episode 6
Unlikely in a healthy non-smoker, but not impossible. This was ruled out after my first biopsy so that was reassuring.
Leukemia: 1 in 14,000 / season 6, episode 10
Langerhans cell histiocytosis: 1 in 560,000 / season 3, episode 10
Wegener’s Granulomatosis: 1 in 100,000 / season 7, episode 23
Blastomycosis: 1 in 100,000 / season 5, episode 20 and Histoplasmosis: 1 in 30,000 / season 3, episode 21
The fungal infections were among the more “common” disorders on the list. AND I had a slight conversion to posistive for “blasto” in my lab work. In November, prior to a lot of my symptoms, I made Christmas wreaths from Holly trees that were a popular hangout for birds. These fungal infections are indigenous to the area where I live and are associated with bird droppings. This could explain the lung nodules, but not the spleen. The lung nodules would have been labeled an opportunistic infection, but the spleen? Ah yes the spleen- no ideas. But it did make us think that there were two processes going on.
Lymphoma (1 in 25,000)/Splenic lymphoma: (1 in 1,000,000) / not on House MD
Spleen cancer is rare and it usually isolates to just the spleen. However, the cancer should show up in my bone marrow or blood (would not explain the lung nodules).
Rosai-Dorfman: (1 in 200,000)/Erdheim-Chester: (<1 in 1,000,000) / season 2, episode 17
Hemophagocytic Lymphohistiocytosis (HLH): 1 in 1,000,000 / too rare for House MD
My lung biopsies did not show the pathology of this disorder, but they did show a lot of benign histiocytes. The laboratory markers were also too low (ferritin, LDH and triglycerides). So probably not.