Myeloproliferative Disorders Flashcards
Name the four types of myeloproliferative disorders
Polycythemia Vera
Myeloid Metaplasia + Myelofibrosis
Essential Thrombocytosis
Chronic Myelogenous Leukemia
how to distinguish P. Vera from other forms of polycythemia
EPO virtually undetectable
Important clinical symptoms common in P vera
Abnormal platelet fxn –> hemorrhage of GI, throat, brain
Peptic Ulcers
Vascular distention of the venous circulation –> cyanosis
Headache, dizziness, melena common
prolonged survival with p. vera can lead to…
- spent phase w/ myeloid metaplasia + myelofibrosis
- Blast cell transformation –> AML
Cause of fibrosis in myelofibrosis
Cytokine (+PDGF+TGFb) release from transformed myeloid cells triggers proliferation of fibroblasts
Myeloid lineage cells that tend to cause myelofibrosis
Megakaryocyte
Common causes of death in Myelofibrosis
Intercurrent infections, thrombotic episodes, bleeding
AML transformaiton
Difference between cells of NHL and MM
MM consist of differentiated B cells
Typically no masses in LN
Five kinds of given Plasma Cell Dyscrasias
Multiple Myeloma Waldenstrom's Macroglobulinemia Heavy Chain Disease Primary Amyloidosis Monoclonal Gammopathy of Undetermined Significance
What are Bence Jones proteins?
The excess heavy or light chains excreted int he urine in Multiple Myeloma
What is multiple myeloma
Plasma cell cancer of the bone marrow – hits skeleton in multiple sites, can go extraosseous.
She also says later its hematopoetic stem cells…not plasma cells…
Bence Jones proteins form casts surrounded by…
Multinucleate giant cells
Some casts have features of amyloid proteins
Main clinical features of Multiple Myeloma
- Infiltration of Bone – Pain+Fractures
- Resorption – Hypercalcemia –> Confusion, weakness, constipation, polyuria, etc.
- Suppressed normal Ig production –> Recurrent Infections
- Hyperviscosity – Hemorrhages, prolonged bleeding, neurologic change
- Renal Insufficenciy (failure in up to 50% - BJ + amyloid)
X ray findings in MM
Sharply punched-out defects ith rounded SOAP BUBBLE appearance on X ray. Maybe generalized osteoporosis
MM is a ___cytic, ____ chromic
normo x2
What is solitary myeloma?
Solitary plasmacytic region in bone or soft tissue
Becomes MM in ones with bone lesions, BUT NOT in non-bone lesions. locally resect those ones.
X ray finding in Waldenstrom’s macroglobulinemia?
none
Problem in Waldenstrom’s macroglobulinemia
Macroglobulins form large aggregates –> hyperviscosity
Symptoms that occur due to hyperviscosity in Waldenstrom’s macroglobulinemia
Visual Impairment (tortuosity of retinal veins
Headaches, Dizziness, Deagness
Bleeding (complexes btw macroglob. and clotting factors)
Cryoglobulinemia (precip at low temp –> Raynauds)
What is gamma Chain disease?
Elderly folks with monoclonal IgG gammopathy
Looks like lymphoma
What is alpha chain disease
Young adults, mediterranean area
Infiltration of lamina propria + LN
Villous atrophy + Malabsorption
When is Mu-chain disease found?
CLL
Most common monoclonal gammopathy?
MGUS
Problem with MGUS?
20% turn into myeloma or Waldenstroms
