Myeloproliferative disorders: Lymphoma

Question 1

What does Myelo mean?

Answer 1

• Originates in the bone marrow or spinal cord

Question 2

What are normal RBC?

Answer 2

• 3-6.5x10^12/l

Question 3

What is normal Hb?

Answer 3

• 11 -18 gram per 100ml

Question 4

What is normal WBC?

Answer 4

4-11x10^9/l

Question 5

What is polycythemia vera- ?

Answer 5

• increased conc of RBC in blood

Question 6

What is Apparent PV ?

Answer 6

• normal red cell count but lower plasma, blood is thicker
• Usually caused by lifestyle, smoking, drinking, and certain meds (diuretics)

Question 7

What is relative PV?

Answer 7

• Similar to apparent but caused by dehydration

Question 8

What is absolute PV?

Answer 8

• an overproduction of red blood cells
• 95% of cases have a genetic link to JAK2 gene leading to uncontrolled production of RBC
• Develops during life and not passed on to offspring

Question 9

What are the symptoms of PV disorders?

Answer 9

• Fatigue
• Trouble breathing
• Itchy skin
• Headache and vision probems
• High blood pressure
• Blockage of vessels / stroke / clots
• bleeding gumps, peptic ulcers
• splenomegaly

Question 10

What is the treatment for PV?

Answer 10

• Not curable
• Watch and wait
• Lifestyle changes
• Lowering the blood count, venesection (1/2 blood taken once a week, then as needed)
• Low dose aspirin
• Control cardio risk factors
• Chemo, mild
• Interferon

Question 11

What is essential thrombocythaemia?

Answer 11

• overproduction of platelets
• associated with JAK2, CALR, MPL gene

Question 12

What are symptoms of thrombocythaemia?

Answer 12

Heart attack/stroke
Headaches, dizziness, migraines, seizures
Burning/ throbbing pain in hands and feet
Bruising/ bleeding
Pain and redness in hands/feet/face, Erythromelalgia
GI bleeding/ blood in urine
Thrombosis- Stroke/heart attack/blurred vision or blindness
Splenomegaly (40-50% at presentation)

Question 13

How is thrombocythaemia treated?

Answer 13

• symptom dependent
• Reduce cardio vascular risks
• Low dose aspirin
• Chemotherapy- Hydroxycarbamine
• Interferon
• Anagrelide- used following other treatment options, a - - drug that inhibits maturation of platelets from
• megakaryocytes
• Phosphorous-32

Question 14

What is primary myelofibrosis?

Answer 14

• overproduction of collagen or fibrous tissue in the bone marrow reducing its ability to produce blood cells
• following reduced blood production, liver and splein take over but not as good
• JAK2, CALR, MPL

Question 15

What are the symptoms of Primary myelofibrosis (Myeloscelerosis)?

Answer 15

Fatigue
Trouble breathing
Anaemia
Weight loss
Fever and night sweats
Abnormal bleeding
Hardening of bone marrow- bone pain
Repeated infections

Question 16

How is Primary myelofibrosis (Myeloscelerosis) treated?

Answer 16

-Treatment, depends on staging
- Early proliferative (cellular) phase
:-Overactive bone marrow, increased megakaryocytes
:-Increase in fibrin in blood- forms blood clots
:-High blood counts
-Fibrotic phase
:-Increase in amount of fibrosis in bone marrow
:-Decrease in blood forming tissue

-As with essential thrombocytosis treatment of symptoms when present
Blood transfusion
Treatment for enlarged spleen- RT, splenectomy
Chemotherapy- hydroxycarbamide
JAK2 inhibitors- can worsen amaemia
Stem cell transplant

Question 17

What are the two types of Hodgkins Lymphoma?

Answer 17

• Classical and Nodular
• Nodular behaves slightly differently

Question 17

What is the main difference between hodgkins and non-hodgkins?

Answer 17

• Hodgkins follows contiguous spread
• Non-hodgkins does not follow contiguous spread

Question 18

What are CD15 and CD30?

Answer 18

• CD15 is seen in 75-85% of CHD cases
• a carbohydrate used in diagnosis of HL
• Present on neutrophils, eosinophils and some monocytes
• Present on Reed-Sternberg cells
• Mediates phagocytosis and chemotaxis
• CD30 = almost all CHD
• Lymphocyte activation antigen
• Member of tumour necrosis factor
• overexpression = molecular basis for growth and cytokine expression that causes hodgkins lymphoma

Question 19

How is HL diagnosed?

Answer 19

• Physical examination
• medical and family history
• FBC
• Blood chemistry
• Lymph node biopsy
• bone marrow biopsy
• imaging, PET CT

Question 20

What are HL signs and symptoms?

Answer 20

Persistent fever
Night sweats
Unintentional weight loss
Persistent cough
All over itching
Pain at site of nodal involvement following alcohol consumption
Palpable node
- if bone marrow involvement
Persistent tiredness/ fatigue
Increased risk of infection
Excessive bleeding

Question 21

How is HL staged?

Answer 21

A= asymptomatic
B= symptoms other than lymphadenopathy present, i.e. Weight loss, night sweats
E= extra nodal disease
S= spleen involvement
X= Bulky >10cm or mediastinal mass> 1/3 of chest diameter

Question 22

How are NHL classified?

Answer 22

• B-Cell lymphoma (most common), Agressive and often include external nodal organs
• Follicular cell, five year survival is 85%
• Mantle cell, usually older patients, more common in males, involves bone marrow, spleen and GI system
• Mucosa cell, usually stomach, maybe lung, skin, thyroid, salivary gland or eye
• Burkitts Lymphoma , rare and aggressive, endemic common in africa, associated with HIV.