Dr. Ayesha Warsi

Alright, so now that we've talked about the basic physiology, let's talk about the approach.

While some patients with myelofibrosis may be asymptomatic at the time of presentation, 70-85% will have at least one symptomatic manifestation.

The most common presenting symptoms in primary myelofibrosis are constitutional symptoms, abdominal discomfort or early satiety from splenomegaly, or consequences of cytopenias.

For example, many patients will present with severe fatigue due to inefficient hematopoiesis.

Other systemic presenting symptoms can include fevers, weight loss, night sweats, and bone pain.

Since extramedullary hematopoiesis can occur in almost any organ, rarely symptoms can include those related to the organ affected, such as the heart, lungs, lymph nodes, or GI or GU tracts.

These can include manifestations such as ascites, pleural effusions, or pulmonary hypertension.

Imagine a patient that presents to the emergency department with a six-month history of severe fatigue.

He is noted to have hepatosplenomegaly on examination.

Initial investigations demonstrate a low hemoglobin of 68, elevated leukocytes of 12, and elevated platelets of 464.

Looking back, you can see that his hemoglobin has been gradually declining over the last few years.

This patient's presentation with anemia, leukocytosis, and thrombocytosis associated with hepatosplenomegaly yields a broad differential.

However, a good history and physical examination will guide your differential diagnosis and may raise flags that prompt you to consider primary myelofibrosis.

Your first step in any patient encounter will be to assess whether your patient is stable or not.

What is their GCS?

Are their ABCs stable?

What are their vitals?

Though rare, critical cases of primary myelofibrosis can have patients presenting with respiratory distress or cardiac tamponade due to extramedullary hematopoiesis that has been occurring in the heart or lungs.

Once your patient is stable, you can move forward with your assessment.

On history, as mentioned already, systemic symptoms may include severe fatigue, fevers, or night sweats.