What Does an Old Fable Have in Common with Primary Myelofibrosis?

There’s an old fable that reminds me, in a way, of primary myelofibrosis, or PMF:

A shepherd is in the desert with his camel, and as night approaches, he pitches his tent, and builds a small fire. After eating his meager meal and feeding the camel, he retires to the inside of his tent to sleep. A half hour later, the camel sticks his nose into the tent and says, “Won’t you let me sleep in the tent with you? It’s very cold out here.” (Hey, it’s a fable…camels can talk.) The shepherd says, “No, camel. Go to sleep where you are.” A half hour later, the camel sticks his head into the tent and repeats his question. Again, the shepherd says no dice. By morning, however, as the sun comes up, the camel is sleeping snugly inside the tent, and the shepherd is shivering outside the tent.

So what does this have to do with PMF?

Glad you asked. PMF is a blood cancer where scar tissue forms in the bone marrow. Abnormal stem cells overproduce mature cells so quickly that the normal blood cells get pushed aside, like the camel pushing the shepherd. When the bone marrow is completely overwhelmed, the process of manufacturing blood cells shifts over to the spleen. You can imagine how happy the spleen is about its new job. Not very. As a result, it enlarges sometimes to more than twice its normal size. In about 50% of reported cases, the patient has a mutation in the JAK2 gene.

PMF is diagnosed through gene mutation analysis of blood cells, blood tests (a CBC panel), bone marrow biopsy, and ultrasound imaging. Symptoms include fever, weight loss, bone pain, gout, abnormal liver function; there are other symptoms, but these are the most common.

Treatment varies from patient to patient, and depending upon the severity of the case, some people may be largely asymptomatic, while in others the disease progresses more quickly.

Erica Zahn

Erica Zahn

Erica Zahn is passionate about raising awareness of rare diseases and disorders and helping people connect with the resources that may ease their journey. Erica has been a caregiver, and is a patient, herself, so she completely relates to the rare disease community--on a deeply personal level.

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