Heme - Myoproliferative disorders Flashcards
(16 cards)
A group of disorders characterized by a proliferation of normally developed (nondysplastic) multipotent hematopoietic stem cells from the myeloid cell line.
Myeloproliferative neoplasms
What are the myeloproliferative disorders?
- essential thrombocythemia
- polycythemia vera
- primary myelofibrosis
- chronic myeloid leukemia
All myeloproliferative disorders can potentially progress to
acute myeloid leukemia
All the myeloproliferative disorders except CML have a varying degree of this mutation
JAK2 mutation
Isolated increased platelets (thrombocythemia) and large hypo granular platelets on peripheral smear
essential thrombocythemia
Increased Hgb, Hct and red cell mass as a result of increased RBC, decreased EPO, normal morphology on peripheral smear
polycythemia vera
Palpable splenomegaly, decreased Hgb, increased WBC and LDH, leukoerythroblastosis and dacrocytes (teardrop cells) on peripheral smear
primary myelofibrosis
Splenomegaly, increased WBC, increased platelets, decreased RBC, decreased leukocyte alkaline phosphatase, increased basophils on peripheral smear.
chronic myeloid leukemia
A peripheral blood smear finding of granulocytes that have reduced segmentation (bilobed neutrophils) caused by myelodysplastic syndrome.
Pseudo-Pelger-Huet abnormality
Clinical features of polycythemia vera
- often asymptomatic
- hyperviscocity syndrome
- plethora
- pruritus
- erythromelalgia
Triad of mucosal bleeding, neurological symptoms and visual changes
hyperviscocity syndrome
Flushed face with a purple hue and cyanotic lips
plethora
Itching that typically worsens when the skin comes into contact with warm water
aquagenic pruritus
A rare condition characterized by intermittent hyperperfusion of the feet and hands that is associated with intense burning pain, erythema, warmth and swelling.
Erythromelalgia
Best initial treatment for polycythemia vera
phlebotomy
Patients with polycythemia vera are at increased risk for
thrombosis and bleeding
