Myeloma and Lymphoma

Question 1

PCV: polycythemia vera

Answer 1

no erythropoitin elevation
an issue of bone marrow- RBC precursor divides autonomously

WBC and PLT may also be elevated, as they also come from myeloid stem cell precursor

you do NOT see lymphocytes

RF
age 50-60, though children and young adults can be affected

can become malignant

si/sx associated with hyperviscous blood

Visual disturbances: blurred vision, amaurosis fugax, scintillating scotoma, ophthalmic migraine

Thrombosis (15%): stroke, MI or angina, claudication, DVT or PE, Budd- Chiari syndrome, superficial thrombophlebitis

Erythromelalgia- burning pain in hands an feed with erythema, pallor, or cyanosis

Pruritis (especially after a warm bath)

Facial plethora

Hepatosplenomegaly

Elevated H/H and red cell mass, basophilia, leukocytosis (40%), thrombocytosis (60%)

Treatment:
1. phlebotomy to keep HCT below 45% in men, 42% in women
This induces a desirable IDA (do not supplement iron)

2. Add hydroxyurea if at high risk for thrombosis (over age 70, prior thrombosis, PLT> 1,500,00, or presence of cardiovascular risk factors)
3. Aspirin every day to help prevent thrombosis (MI, CVA, PE, DVT)
4. IFN-alpha if there is refractory pruritis or refractory erythrocytosis
5. If there is symptomatic hyperuricemia, give allopurinol 300mg every day

Question 2

Polycythemia (erythrocytosis) causes

Answer 2

Hypoxemia: MCC of increasd RBC production

• COPD
• OSA
• Living at high altitude

inappropriate increase in erythropoeitin
pheochromocytoma
RCC
HCC
Hemangioblastoma

Question 3

Multuple myeloma

back pain
hypercalcemia
anemia
renal failure

Answer 3

malignancy of plasma cells
fully matured B cell that makes abs.

most common bone marrow cancer in adults

protein electrophoresis shows IgG monoclonal single spike, M spike
and Bence Jones IgG light chains

Clinically:
back pain
radicular pain
weakness, fatigue, weight loss
constipation
pathologic fractures
pallor
bony tenderness
frequent infections

Labs: cells in the bone marrow interfere with other cell lines

anemia, decreased WBCs
renal insufficiency (casts plug up the kidney= myeloma kidney)
hypercalcemia (ams, constipation)- local osteolytic factors

SPEP: M protein
UPEP: Bence- Jones protein (note- this won’t show up on UA)

Radiology: punched out lytic lesions

Plasma cells (clockface chromatin) dense within LNs on bx, with cleared out cytoplasm

Tx:chemo/rads/bone marrow transplant
treat fractures and infections
supportive care, as prognosis is poor (a couple years after diagnosis)

Complications:
spinal cord compression

Skeletal survey:

• xrays of the entire skeleton to look for lytic lesions
• SPEP and UPEP to look for M- spike and Bence Jones

Question 4

Lymphoma

Answer 4

solid lymphoid malignancy
definite lymphoid mass as opposed to myeloma or leukemia

40 subtypes: Hodgkin and non-Hodgkin

Question 5

Hodgkin lymphomas

Answer 5

There are 4 types.
All are B cell lymphoma
Nodular sclerosis is the most common (70%)

painless cervical lymphadenopathy

mediastinal lymphadenopathy on xray

B symptoms: fever, weightloss, night sweats

pruritis
hepatosplenomegaly

Bx: Reed- sternberg cells against a background of reactive tissue. They shrink a little bit, and a clear area surrounds them

Tx: radiation and chemotherapy

Prognosis is pretty good

Question 6

Nodular sclerosis

Answer 6

Nodular sclerosis is the most common (70%)
nodules surrounded by sclerotic bands of collagen

W=M
Bimodal age distribution (20, 65)

Question 7

Non-Hodgkin lymphoma

Answer 7

MC:
-Diffuse large B cell
-Follicular small cell, t(14,18)
cleaved cells, notched and dented- appearing

-Small lymphocytic- same disease as CLL, but with malignant cells found in the lymph nodes rather than bone marrow
-Burkitt lymphoma
EBV and t(8,14)
Starry sky appearance

H and P:
-Painless generalized lymphadenopathy

Prognosis is worse than Hodgkin lymphoma

Question 8

How to diagnose MM?

Answer 8

SPEP- monoclonal ab spike (M protein)
UPEP- Bence- Jones proteins
Bone marrow biopsy- increased plasma cells

Question 9

Associated with EPV

Answer 9

Burkitt lymphoma

Question 10

Reed- Sternberg cells, cervical lymphadenopathy, night sweats

Answer 10

Hodgkin lymphoma

Question 11

Bence-Jones proteins, osteolytic lesions, high calcium

Answer 11

Multiple myeloma

Question 12

Translocation 14;18

Answer 12

Follicular lymphoma

Question 13

Most common lymphoma in the US

Answer 13

Diffuse large B cell lymphoma

Question 14

Translocation 8;14

Answer 14

Burkitt lymphoma

Question 15

Most common form of Hodgkin lymphoma

Answer 15

Nodular sclerosing Hodgkin lymphoma

Question 16

“starry- sky pattern” due to phagocytosis of apoptotic tumor cells

Answer 16

Burkitt lymphoma

Question 17

high HCT/hemoglobin pruritis (Especially after hot bath or shower), burning pain in hands or feet (erythromelalgia)

Answer 17

polycythemia vera