A cachectic 65yo woman was presented to her GP with lethargy, fatigue & vague abdominal pain. The GP noticed she had multiple bruises, but the patient explained she had always been clumsy & knocked against furniture frequently. The GP wasn’t convinced so performed quick iSTAT blood test & noticed her Hb 68. He referred the patient to ED.
In ED she was noted to have marked splenomegaly.
Hb 67, WCC 18, plts 83, mild transaminitis on LFT’s.
This is her CT scan: What are the findings? What is the differential diagnosis?
Myelofibrosis is a myelproliferative disorder where bone marrow is replaced with connective tissue & progressive fibrosis.
Characteristically there is extramedullary haematopoiesis (mostly occurs in the spleen) which accounts for the progressive splenomegaly & anaemia. The platelet count is variable. It is more common in older men, & can transform into leukaemia. There can be associated lymphadenopathy, & hepatomegaly with portal hypertension
Myelofibrosis can be a primary disorder (ie. myeloid metaplasia), or secondary due to marrow replacement from malignancy or toxins (such as toluene or benzene) or significant radiation exposure.
The longterm prognosis for patients with myelofibrosis is poor, with an average survival of 2-3yrs after diagnosis.