MLAB 1315- HematologyKeri Brophy-Martinez

Unit 23: CHRONIC MYELOPROLIFERATIVE

DISORDERS (MPD)

CHRONIC MYELOPROLIFERATIVE DISORDERS (MPD)

MPD’s are characterized by a hypercellular bone marrow with increased quantities of one or more cellular lineages in the peripheral blood.

Idiopathic myelofibrosis

Characteristics Marrow fibrosis

90% of attempts result in dry tap. Fibroblasts and increased collagen production lock in

the marrow contents. Extramedullary hematopoiesis or myeloid

metaplasia of spleen and liver NRBC’s and immature WBC’s in the peripheral

blood, teardrop red cells, abnormal platelets

Idiopathic myelofibrosis

Treatment Transfusion for anemia Iron, folate and B12 Steroids Splenectomy BM transplant

Idiopathic myelofibrosis

Prognosis Median survival time is about 5 years from

time of diagnosis.

Polycythemias

Polycythemia vera Stem cell disorder characterized by a remarkable

increase in red blood cell mass and total blood volume. There is also an increase in myeloid and megakaryocytic elements in the bone marrow.

Polycythemia vera

Clinical features Patients have a ruddy cyanotic complexion due to

congestion of blood vessels. Itching Fever and night sweats Splenomegaly Brain circulatory disorders and TIA’s Onset is usually around 60 years of age.

Polycythemia vera

Laboratory features Increased red cell mass with Cr51

Increased H&H, WBC, plts

Polycythemia vera

Treatment Therapeutic phlebotomy for rapid reduction of

RBC mass. Radioactive phosphorous for myelosuppression.

Prognosis Survival time from diagnosis is 8-15 years 10-15% of patients convert to acute

nonlymphocytic leukemia.

Secondary polycythemias There are many causes that all result in increased

secretion of erythropoietin Physiologically appropriate increase in erythropoietin

High altitude Chronic pulmonary disease Obesity/sleep apnea

Defective oxygen transport Smoking High oxygen affinity hemoglobinopathies

Physiologically inappropriate increase in erythropoietin Renal disease Neoplasms Endocrine disorders

Relative polycythemia

Stress polycythemia Dehydration causes decrease plasma volume

Essential thrombocythemia

Rare chronic MPD in which platelets are increased and function is abnormal.

Platelet count is > 600,000 x 109/L