【CSCO 2022 Preview】Focus on young patients with myeloproliferative tumors

Bone marrow is found in many bones in our body and is the largest hematopoietic organ in the human body, divided into red bone marrow and yellow bone marrow
.
The bone marrow that can produce blood cells is slightly red, called red bone marrow, and the bone marrow of fetuses and infants is red bone marrow
.
With age, red bone marrow is gradually replaced by yellow bone marrow, and adult red bone marrow and yellow bone marrow account for about half
.
When the body is severely ischemia, part of the yellow bone marrow can be transformed into red bone marrow and restore the ability to
make blood.
Healthy bone marrow is like a factory, which can generate various blood cells hematopoietic stem cells, which are released into the peripheral blood cells through differentiation, such as red blood cells, white blood cells, platelets, etc.
, and perform their respective tasks until aging and death, this process is constantly cyclically renewed
.

Myeloproliferative tumors (MPNs), formerly known as myeloproliferative disorders (MPDs), are a clonal type of stem cell disease that manifests as the proliferation of excessive mature cells
in the bone marrow.
The clinical manifestations are one or more blood cells in the peripheral blood, often accompanied by hepatic and splenomegaly, bleeding tendency, thrombosis, and extramedullary hematopoiesis
.
It is more common in middle-aged and elderly people between 50~70 years old, and often dies
in the terminal stage due to myelofibrosis, systemic failure or transformation into acute leukemia.

According to the 2016 WHO classification of myeloproliferative neoplasms (MPNs), there are 7 types, of which polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF) are considered to be typical myeloproliferative tumors (MPNs).

Polycythemia vera (PV) is a clonal myeloproliferative tumor (MPN) originating from hematopoietic stem cells, with an annual incidence of 0.
4~2.
8/100,000.

PV patients are mostly red on the face and lips, which are prone to thrombosis and increased
blood pressure.
Blood routine often shows markedly elevated
hemoglobin and hematocrit (HCT).

Primary thrombocythemia (ET) has a slow course, many patients are asymptomatic for a long time, and the main clinical manifestations of the disease are bleeding and thrombosis
.
Unlike other myeloproliferative diseases, fever, sweating, weight loss, etc
.
are very rare.

Myelofibrosis (MF) is a stem cell clonoproliferative disease
characterized by excessive secretion of cytokines that promote fibrous tissue proliferation, extramedullary hematopoiesis, and transformation into acute leukemia.
Most of the disease has a slow onset, and there may be no symptoms in the early stage, and then gradually appear fatigue, night sweats, palpitation, shortness of breath and other weak symptoms and abdominal pain, abdominal mass, bone pain, etc
.

In fact, the annual incidence of MPN patients is relatively low, which is rare in all hematological tumor diseases, and its annual incidence is 6 ~ 10 per 100,000
.
Most of these patients are older or older (60~65 years old)
when they are diagnosed.
However, a large number of research reports in China show that the diagnosis age of MPN patients is relatively young (even 5~10 years younger).

About 20% of all patients diagnosed with MPN are of childbearing age, so they need to face pregnancy problems
.
Especially patients with essential thrombocythemia are younger, and the peak age of diagnosis is in the reproductive age, plus there are more women (about 60%)
.
Therefore, although the incidence of MPN patients is small, it is worth paying attention
to this part of patients of childbearing age.
Why is it worth paying attention to? First, with the improvement of medical diagnosis level and the deepening of understanding of molecular testing, coupled with many methods such as physical examination, most patients can be diagnosed
at an early stage.
Second, most people in contemporary society have been moving their childbearing age backward, so childbearing will be late
.
Third, China's open two-child policy has given many patients the opportunity to
get pregnant.
Therefore, this is a problem
that we must face.

On November 11, 2022, the 25th National Congress of Clinical Oncology and the 2022 CSCO Annual Conference invited Professor Jiang Qian from Peking University People's Hospital to give a detailed interpretation
of the management of young myeloproliferative tumor patients.

Resources:

https://mp.
weixin.
qq.
com/s/ezOs7OyUyjtzTmDRZyW8ug

https://mp.
weixin.
qq.
com/s/u3Fn9vg2UXaew49BXCOSMw

https://mp.
weixin.
qq.
com/s/So89sLHqhgN-9HkAQ9n5lA