Myeloproliferative Disorders

Question 1

Name the four types of myeloproliferative disorders

Answer 1

Polycythemia Vera
Myeloid Metaplasia + Myelofibrosis
Essential Thrombocytosis
Chronic Myelogenous Leukemia

Question 2

how to distinguish P. Vera from other forms of polycythemia

Answer 2

EPO virtually undetectable

Question 3

Important clinical symptoms common in P vera

Answer 3

Abnormal platelet fxn –> hemorrhage of GI, throat, brain
Peptic Ulcers
Vascular distention of the venous circulation –> cyanosis
Headache, dizziness, melena common

Question 4

prolonged survival with p. vera can lead to…

Answer 4

• spent phase w/ myeloid metaplasia + myelofibrosis

- Blast cell transformation –> AML

Question 5

Cause of fibrosis in myelofibrosis

Answer 5

Cytokine (+PDGF+TGFb) release from transformed myeloid cells triggers proliferation of fibroblasts

Question 6

Myeloid lineage cells that tend to cause myelofibrosis

Answer 6

Megakaryocyte

Question 7

Common causes of death in Myelofibrosis

Answer 7

Intercurrent infections, thrombotic episodes, bleeding

AML transformaiton

Question 8

Difference between cells of NHL and MM

Answer 8

MM consist of differentiated B cells

Typically no masses in LN

Question 9

Five kinds of given Plasma Cell Dyscrasias

Answer 9

Multiple Myeloma
Waldenstrom's Macroglobulinemia
Heavy Chain Disease
Primary Amyloidosis
Monoclonal Gammopathy of Undetermined Significance

Question 10

What are Bence Jones proteins?

Answer 10

The excess heavy or light chains excreted int he urine in Multiple Myeloma

Question 11

What is multiple myeloma

Answer 11

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…

Question 12

Bence Jones proteins form casts surrounded by…

Answer 12

Multinucleate giant cells

Some casts have features of amyloid proteins

Question 13

Main clinical features of Multiple Myeloma

Answer 13

1. Infiltration of Bone – Pain+Fractures
2. Resorption – Hypercalcemia –> Confusion, weakness, constipation, polyuria, etc.
3. Suppressed normal Ig production –> Recurrent Infections
4. Hyperviscosity – Hemorrhages, prolonged bleeding, neurologic change
5. Renal Insufficenciy (failure in up to 50% - BJ + amyloid)

Question 14

X ray findings in MM

Answer 14

Sharply punched-out defects ith rounded SOAP BUBBLE appearance on X ray. Maybe generalized osteoporosis

Question 15

MM is a ___cytic, ____ chromic

Answer 15

normo x2

Question 16

What is solitary myeloma?

Answer 16

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.

Question 17

X ray finding in Waldenstrom’s macroglobulinemia?

Answer 17

none

Question 18

Problem in Waldenstrom’s macroglobulinemia

Answer 18

Macroglobulins form large aggregates –> hyperviscosity

Question 19

Symptoms that occur due to hyperviscosity in Waldenstrom’s macroglobulinemia

Answer 19

Visual Impairment (tortuosity of retinal veins
Headaches, Dizziness, Deagness
Bleeding (complexes btw macroglob. and clotting factors)
Cryoglobulinemia (precip at low temp –> Raynauds)

Question 20

What is gamma Chain disease?

Answer 20

Elderly folks with monoclonal IgG gammopathy

Looks like lymphoma

Question 21

What is alpha chain disease

Answer 21

Young adults, mediterranean area
Infiltration of lamina propria + LN
Villous atrophy + Malabsorption

Question 22

When is Mu-chain disease found?

Answer 22

CLL

Question 23

Most common monoclonal gammopathy?

Answer 23

MGUS

Question 24

Problem with MGUS?

Answer 24

20% turn into myeloma or Waldenstroms