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Haematology > Haematological Malignancy in Practice > Flashcards

Haematological Malignancy in Practice Flashcards

1
Q

54 year old scaffolder

Tiredness progressively worse over last 2-3 weeks

What other symptoms should you ask about?

A
  • SOB, dizziness, palpitations – anaemic symptoms
  • Bleeding, diet - ?cause of anaemia
  • Mood, appetite, sleep pattern, motivation - ?depression
  • Weight gain, constipation, skin and hair changes - ?hypothyroidism
  • Fevers, sweats, weight loss - ?infection, ?malignancy
  • Systemic enquiry
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2
Q

Further questioning:

Over last 2 weeks - SOB and dizzy on climbing scaffold

Feeling hot and cold

Coughing up green phlegm

Examination:

Very pale

Temp 38.5

Dull percussion note R base with decreased air entry

Petichiae around ankles

what investigaitons would you do?

A

—FBC, film

—Coagulation screen

—Haematinics – B12, folate, ferritin

—U&Es, LFTs, CRP

—Sputum culture

—CXR

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3
Q

FBC Result:

Blood count shows he is very anaemic

Normal MCV and WCC

Very neutropenic

Normal reticulocyte count

Signs of significant bone marrow failure

what are some differential diagnosis?

A

—A – Acute leukaemia

—B – Aplastic anaemia

—C - B12 deficiency

—D – Metastatic ovarian cancer

—

—Why or why not these conditions?

Could be B but most likely A as normal reticulocyte count

C not likely as MCV is normal

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4
Q

what is his diagnosis?

Blood film shows circulating blast cells

A

—Acute myeloid leukaemia (AML)

—

—Short history

—Uncontrolled proliferation of immature myeloid cells therefore acute

This guy would be brought in and treated his acute sepsis with antibiotics and fluids etc, would be transfused as symptomatic anaemia and bleeding then would be diagnosed and treated his leukaemia with chemo and bone marrow as he is fit

Died post bone marrow transplant of graft vs host disease

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5
Q

20 year old student

—Presents with 1 month history of gradually increasing neck swelling

—Weight loss of half a stone

—Generalised itch

—Night sweats

—O/E 2x3 cm cervical lymphadenopathy

—Also axillary lymphadenopathy

—FBC – normocytic normochromic anaemia, eosinophilia

what are some differential diagnosis?

A

Hodgkin’s Lymphoma

Non-Hodgkin’s lymphoma

Acute lymphoblastic leukaemia

Metastatic non haematological malignancy

Infections:

  • viral – EBV, CMV, HIV
  • Bacterial – draining, local lymph nodes
  • TB, toxoplasma
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6
Q

whata re some fither invesitgations you could do?

A

—Lymph node biopsy

—Virology (particularly HIV)

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7
Q

what is the presentation of a lymphoma?

A

—Lymphadenopathy

—Splenomegaly +/- hepatomegaly

—B symptoms:

  • —fever >38oC
  • —weight loss >10% body weight in 6 mths
  • —night sweats

—Symptomatic anaemia or other cytopenias

Biopsy is critical in making the diagnosis

—

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8
Q

what is the diagnosis?

A

—Staged by PET-CT scan

—Stage IIB

—Combination chemotherapy - ABVD

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9
Q

75 year old man:

—Intermittent claudication for 2 years

—Admitted with painful toe

—Hb 159 120-160

—MCV 74 82-99

—RCC 6.2 3.8-5.0

—WBC 11 4-10

—Plts 850 140-400

Microcytic

Slightly leucocytosis and thrombocytosis

—What are the causes of high platelets?

A

—Infections

—Post surgery / trauma

—Malignancy

—Iron deficiency

—Inflammation – IBD, Rheumatoid arthritis

—Primary myeloproliferative disorder

Vast majority of thrombocytosis is a reactive thing - About anything can cause secondary thrombocytosis

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10
Q

what investigations would you do for thrombocytosis

A

—FBC

—Blood film

—Inflammatory markers (ESR, CRP)

—Ferritin (is he iron deficienct?)

—JAK2, CALR & MPL mutation screening - Often can make a diagnosis by looking at these mutations and looking at the case as a whole

—Consider BCR-ABL

—Other investigations if clinical suspicion of malignancy

—Bone marrow examination not first line

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11
Q

Results:

—ESR 24mm/hr, CRP <4

—Ferritin 3 - iron deficient

—Positive for JAK2 V617F mutation (one of the hall mark mutations for myeloproliferative disease)

What is the diagnosis?

A

Not 2 as CRP is normal

Seems like it is a vascular event and doesn’t seem to be any secondary infection

4 is also possible but presentation is not typical of this as normally you see splenomegaly and low counts

5 you would see microcytosis, raised red cell count but not in a 75 year old man as would of presented way before that and you wouldn’t see iron deficiency

So why is it not 3 – one of the hallmark features of PV is that they are iron deficient, they burn out their iron stores

3 is an isolated high platelet count where as in 1 the dominant thing is polycythaemia

You can see high platelets in PV but If you have polycythaemia then you have PV but if you just have high platelets then its just ET

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12
Q

75 year old man:

—Iron deficient Polycythaemia Vera

—Myeloproliferative disorder

what is his treatment?

A

—Aspirin (antiplatelet), Venesection (remove blood), Hydroxycarbamide (inhibits production in bone marrow reducing platelet count)

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13
Q

55 year old man:

—Low back pain – progressively worse

—Tired last few months

—Previously fit and well

—No weight loss

Very common symptoms - And often not due to serious pathology

what are some key questions for back pain?

A

—Duration

—Getting worse?

—Severity – interfering with sleep?

—Radiation

—Leg weakness or numbness?

—Bladder or bowel disturbance?

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14
Q

what invesitgations may you do?

A

—FBC, ESR

—U&E, Ca, Phos, Immunoglobulins & protein electrophoresis

—Bence Jones protein (or serum free light chains)

—Xray of spine

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15
Q

what is seen on invesitgaiton?

A
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16
Q

what is the diagnosis?

A

—Myeloma

—Malignancy of plasma cells

17
Q

what is the treatment of a myeloma?

A

—Local – surgical decompression or radiotherapy

—Systemic – induction chemotherapy (various regimens)

—Bone protection (IV bisphosphonate (Bisphosphonate switch of osteoclasts and in myeloma they switch osteoclasts on and cause release of calcium into the bloodstream) Zolendronic acid)

—Consolidation (autologous stem cell transplant

—Maintenance (in clinical trial only in UK at present)

18
Q

83 year old woman:

—Admitted for bladder repair; denies other symptoms

—On examination-marked splenomegaly

—What might be the cause?

A

—Low grade lymphoma

—Chronic leukaemias (CML, CLL)

—Myeloproliferative disorders

—Portal hypertension / liver disease

—Infiltration from sarcoidosis, other malignancies

—Infections eg chronic malaria, visceral Leishmaniasis

(Don’t see in acute leukaemia as mainly in bone marrow and happens rapidly so spleen doesn’t have time to response)

19
Q

83 year old woman

—Blood count – Hb 100, MCV 96, WCC 1.2, Neuts 0.5, Plts 58

Neutropenic

Thrombocytopenic

Tear drops can be seen - associated with many conditions

In D you can see blast cell which shouldn’t be in peripheral blood, also red cell in C in peripheral blood which shouldn’t be there so should think is the bone marrow chucking them out because it is so full?

What is the diagnosis?

A.Chronic myeloid leukaemia

B.Myelofibrosis

C.Metastatic breast cancer in bone marrow

D.Chronic malaria

A

Giant platelets let us know it is B

  • Leukoerythroblastic blood film with teardrop poikilocytes, blasts and giant platelets
  • Extensive reticulin fibrosis in bone marrow
20
Q

whata re the features of myelofibrosis?

A

—De novo and transformed - Can arise on its own or transform form other myeloproliferative conditions like PV or ET

—Splenomegaly (can be massive)

—Symptoms from cytopenias or spleen

—Weight loss, extreme tiredness

—Leukoerythroblastic blood film (things form bone marrow spilling into peripheral blood), teardrop red cells

—Marrow fibrosis – Reticulin stain (Key features if fibrosis in bone marrow

—Most positive for one of JAK2/CALR/MPL mutations - Most of these people with have an identifiable mutation from myeloproliferative disease

21
Q

what is the treatment of myelofibrosis?

A

Treatment very varies and same with prognosis—

Transfusion, hydroxycarbamide, thalidomide, JAK2 inhibitors (Ruxolitinib), allogeneic stem cell transplantation

Ultimately allogenic stem cell transplantation is the curative treatment

Allografting is high risk so need to make sure risk of disease and symptoms outweigh the transplant

22
Q

72 year old woman:

Blood count prior to elective cholecystectomy

Otherwise asymptomatic

Hb 120g/l (normal)

WCC 38.1

Lymph 34.7

Neut 3.1

Platelet 230

Raised WCC consisting of lymphocytes

Possible causes of lymphocytosis?

A

—Viral infection (e.g. EBV, CMV, HIV) – too high for this

—Other infections – TB, brucellosis, syphilis

—Vasculitis

—Acute lymphoblastic leukaemia

—Chronic lymphocytic leukaemia

—Lymphoma

23
Q

Blood film:

—Small, mature, homogenous lymphocytes

—“smear” cells (cells broken down, don’t see in normal lypmhocytes)

—Red cells, platelets normal

All look like the same

what are osme further tests?

A

—(1) Flow cytometry

—Cells labelled by monoclonal antibodies conjugated to fluorochromes directed against cell surface markers (tells you if B or T cells and if they carry abnormal markers)

—

—(2) Look for underlying cause

—EBV, CMV, HIV serology

—Brucella, syphilis serology

—Monospot test

24
Q
A

Cells are small mature, no bone marrow failure, lymphoid in nature

CLL usually causes a peripheral blood leucocytosis

25
Q

what is the treatment of this persons Chronic lymphocytic leukaemia?

A

—Early stage, asymptomatic

—Treatment – nothing (watch and wait)

26
Q

1 year later….

—Fatigue, pallor, shortness of breath

—FBC

—Hb 77 g/l

—MCV 95fl

—WCC 40.1

—Lymph 35.6

—Neut 4.0

—Platelet 370

Significant anaemia

Doesn’t have bone marrow failure

Possible explanations?

A

—Blood loss

—Iron/ B12/ folate deficiency

—Bone marrow failure due to CLL

—Autoimmune haemolysis

—Something else (e.g. renal failure)

27
Q

Blood film:

Lymphocytosis (as before)

Spherocytes

Polychromasia (cells that contain RNA, last stage of maturation), nucleated red cells (sign of marrow is stressed and spitting out immature red cells or there is an infiltrative process)

A

Further tests:

Direct anti-globulin test – positive for IgG

LDH, bilirubin – raised

Reticulocyte count - raised

28
Q

Doesn’t have bone marrow failure as other counts are okay

She has significant anaemia, spherocytosis, increased polychromasia so may be bleeding like a upper GI bleed

You see spherocytes in autoimmune haemolysis

A

Autoimmune haemolysis

Positive DAT – direct antiglobulin test, same as coombs test – all your doing is seeing if there is coating of the red cells with antibody so you use a reagent that binds human antibody and it causes red cells to stick together

29
Q

what is the treatment of autoimmune haemolysis?

A

Steroids

Folic acid (need smore fuel as making excess red cells)

If not responding, therapy that treats both autoimmune haemolysis and CLL (e.g. rituximab – knocks out antibody production)

CLL is driving this process - Typically we give chemotherapy to treat CLL to remove the cause and it also indirectly treats the haemolysis

Haematology (15 decks)

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