Chronic Myeloproliferative disorders Flashcards
Teardrop cell (Darcocytes)
Dry Tap (fibrosed BM)
Splenomegaly
+/- JAK2 mutation
Myelofibrosis
My fine brown eyes are tapped dry of tears over Jak.
Itching after shower JAK2 mutation (Acquired)
Polycythemia Vera
______ occur when the bone marrow, the spongy tissue inside the large bones in the body, makes too many blood cells.
These cancers are sorted based on which type of blood cell is overproduced:
red blood cells, white blood cells, or platelets.
Sometimes the body will make too many of more than one type of blood cell, but usually one type is affected more than the others.
Myeloproliferative neoplasms (MPN)
aka
Chronic Myeloproliferative disorders
A myeloid stem cell develops into what 3 things
RBC, WBC, Platelet
With the exception of chronic myelogenous leukemia, the chronic myeloproliferative disorders (especially polycythemia vera) frequently harbor a mutation in the _________.
This mutation results in _________.
non-receptor cytoplasmic tyrosine kinase, Janus kinase 2 (JAK2).
Results in constitutive tyrosine phosphorylation activity, and consequently, activation of the STAT pathway
Elevated RBCs, WBCs, Platelets
Low EPO
JAK +
Polycythemia Vera
Thrombocytosis (elevated platelets)
+/- JAK2 mutation
Essential Thrombocytosis
Low RBCs
+/- JAK2 mutation
Myelofibrosis
Low RBCs
Elevated WBCs & Platelets
JAK –
CML
JAK – = no JAK mutation
Erythromyalgia:
severe, burning pain and red/blue discoloration of extremities due to episodic blood clots in vessels
Rare.
Polycythemia Vera
or
Essential Thrombocytosis
Massive proliferation of Megakaryocytes
+/- abnormally shaped platelets
Essential Thrombocytosis
Elevated Megakaryocytes
Extramedullary Hematopoesis –> Splenomegaly
Fibrosed Bone Marrow
Myelofibrosis
