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Haem Flashcards

1
Q

Precipitants of VTE

A
  • accumulation of activated factors
  • increased platelet adhesion
  • increased leukocyte migration and adhesion
  • hypoxia
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2
Q

Antidote to heparin

A

Protamine sulphate

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3
Q

Monitoring of unfractionated heparin

A

Via aPTT or anti-Xa assay

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4
Q

Monitoring of warfarin

A

INR <2.5 if first episode, AF or cardiomyopathy

INR <3.5 if recurrent DVT, mechanical valve, graft or antiphospholipid syndrome

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5
Q

A 74 year old woman on regular monitoring for warfarin therapy has a sudden increase in her INR from 2.2 to 5.0. What would be your immediate management?

A

Large increase in INR

  • stop warfarin
  • give vitamin K
  • check INR daily until normal
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6
Q

An 80 year old man on warfarin therapy for AF presents to A&E after a fall. On investigation there is evidence of severe internal bleeding and he will need to be transferred to theatre asap. What is your initial management?

A
  • give prothrombin complex concentration or FFP

- give IV vitamin K

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7
Q

What are the main clinical features of an acute leukaemia?

A
  • rapid onset
  • > 20% blast cells
  • BM marrow failure: fatigue, pallor, breathlessness (anaemia), recurrent infections (neutropenia), bleeding (thrombocytopenia)
  • organ infiltration: hepatomegaly, splenomegaly, lymphadenopathy, bone pain, CNS, skin, gum hypertrophy
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8
Q

A 45 year old woman is brought to A&E with a skin infection. She says that she cut her leg whilst gardening yesterday. The area now looks severely infected with some blackened areas. Initial investigations show an Hb of 80 and a high WCC, with granulated cells on a blood film. Cytochemistry with a Sudan black stain and immunophenotyping confirm the diagnosis.

A

AML
This lady is presenting with necrotising fasciitis, caused by an infection with Staph Aureus. Because of her underlying AML she has a high WCC with many immature blast cells causing a functional neutropenia. She is also at risk of developing DIC. Usually Auer rods and granular cells will be seen on blood film but this cannot be relied upon. A combination of cytochemistry and immunophenotyping can confirm the diagnosis.

She will need immediate supportive management then chemotherapy over 6 months to treat the AML.

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9
Q

A 55 year old man with suspected leukaemia is investigated using cytochemistry and immunophenotyping after presenting to the GP with new onset severe epistaxis. On examination he has bleeding gums demonstrating signs of hyperplasia, petechiae, hepatomegaly and splenomegaly. His blood film showed a proliferation of hypergranulated cells with large nuclei and a ‘bundle of sticks’ appearance in the cytoplasm.

What is his diagnosis and what chromosomal abnormality is he most likely to have?

A

Acute promyelocytic leukaemia
T(15;17)
People with APML are prone to DIC. This man has presented with DIC caused spontaneous severe haemorrhage. The cells on the blood film are the characteristic faggot cells seen - the accumulation of Auer rods causes a ‘bundle of sticks’ appearance on visualisation of the cells.

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10
Q

A 3 year old girl presents to A&E in distress. She seems drowsy but when asked about pain she points to her legs. On examination, she is pale, with severe lymphadenopathy and organomegaly. Her mother is concerned that she has been bruising a lot recently. FISH analysis shows that she has the Philadelphia chromosome. What is her diagnosis and what is the most appropriate management?

A

ALL
This girl shows signs of anaemia (pallor, fatigue), organ infiltration (bone pain, organomegaly) and thrombocytopenia (easy bruising). Her severe lymphadenopathy is characteristic of ALL.

She will first need immediate supportive management with blood products , fluid and electrolytes. ALL patients are particularly susceptible to hyperuricaemia, hyperphosphatemia and hyperkalemia. She will then need a chemotherapy regimen inclusive of imatinib as she has the t(9;22) to induce remission. This should be followed up with high dose multi drug chemotherapy and intrathecal CNS treatment. Chemotherapy should be maintained for 2 years (would be 3 if she was a boy).

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11
Q

Supportive management of ALL

A

Treat:

  • Hyperuricaemia: allopurinol, hydration
  • hyperphosphastemia: saline diuresis, phosphate binders (long term)
  • hyperkalemia: 10% calcium gluconate, insulin, 50mls 50% dextrose
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12
Q

Explain how mutations in tyrosine kinases lead to CML.

A

Normal tyrosine kinases transmit cell growth signals to the nucleus.
Abnormal activation leads to an increase in mature cells causing polycythaemia if red cells, essential thrombocythaemia in platelets and CML if granulocytes.

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13
Q

Which mutation is associated with essential thrombocythaemia ONLY?

a) Philadelphia chromosome
b) Calreticulin
c) MPL
d) JAK 2
e) BCR-ABL

A

C)MPL - present in about a quarter of cases of ET

The Philadelphia chromosome is the translocation (9;22) classically associated with CML. Calreticulin mutations can be found in both ET and primary myelofibrosis. JAK 2 has been found to be present in all cases of polycythaemia Vera and 60% of cases of ET and primary myelofibrosis. The BCR ABL fusion gene is associated with CML; the BCR ABL: ABL ratio can be monitored in the treatment of CML.

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14
Q

A 70 year old man presents to his GP after a long history of recurrent headaches. On questioning, he also reports dizziness and some visual disturbances. He also complains of feeling itchy after getting out of the shower. O/E he has retinal vein enlargement and gout in his right foot. How would you confirm the diagnosis and then treat this patient?

A

This man has polycythaemia Vera. He shows signs of hyperviscosity - headaches, dizziness, visual disturbances - and increased histamine release (aquagenic pruritis). These patients often have gout due to hyperuricaemia secondary to increased red blood cell turnover.

Investigations - the patient will have a high haematocrit, Hb and may also have an associated increase in platelet number.

Management - aim to keep HCt below 45% to reduce hyperviscosity and use aspirin if platelets high, to decrease the likelihood of thrombosis. If the PV is severe cytoreductive therapy with hydroxycarbamide can also be used.

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15
Q

A 55 year old woman presents to A&E with bleeding gums. 3 weeks ago she had a stroke and is now experiencing dizziness and headaches regularly. She is now taking aspirin but her platelet count is still high. What further management can be used for this patient?

A

This patient has essential thrombocythaemia. The staple of treatment is to reduce the number of platelets. Low risk patients are often given aspirin. However, when the platelet count is very high, the sequestration of vWF by the high mass of platelets can increase the risk of bleeding and therefore the use of aspirin is counterproductive. This patient needs either anagrelide, which works to inhibit platelet formation, or hydroxycarbamide (hydroxyurea),an antimetabolite which decreases production of deoxyribonucleotides.

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16
Q

A 65 year old man with a history of essential thrombocythaemia presents to his GP with unintentional weight loss, fatigue and might sweats. On examination he has massive organomegaly. A FBC shows a low WBC and high platelet count. A bone marrow aspirate is performed and shows a dry tap with increased collagen fibrosis. What is his diagnosis?

a) Essential thrombocythaemia with prefibrotic changes
b) Transformation to leukaemia
c) ET with secondary PV
d) Secondary myelofibrosis
e) Myelodysplastic syndrome

A

D) secondary myelofibrosis - this man has new myelofibrosis secondary to ET. This is a clinal myeloproliferative disorder with proliferation of megalokaryocytes and granulocytes. It is associated with reactive BM fibrosis (increased collagen and dry tap) and extramedullary haematopoiesis (hence the organomegaly). The organomegaly may also cause Budd Chiari syndrome.

Only 70% of cases of primary myelofibrosis are symptomatic. The disorder can lead to a hypermetabolic state, presenting with weight loss, fatigue, dyspnoea, night sweats and hyperuricaemia. The prefibrotic changes include mild blood changes and hypercellular BM. Fibrotic changes are splenomegaly, a dry tap, collagen fibrosis, osteosclerosis. A blood film will show many blast cells and tear drop poikilocytes.

Unfortunately, the prognosis for myelofibrosis is not good. The median survival is 3-5 years and is even less if there is a severe anaemia, thrombocytopenia or massive splenomegaly.

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17
Q

Which of the following symptoms is not associated with CML?

a) Easy bruising
b) Fatigue
c) Lymphadenopathy
d) Hepatosplenomegaly
e) Gout

A

C) lymphadenopathy - more commonly associated with acute leukaemia

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18
Q

A 53 year old female with known CML presents with a leukocyte count of 150x10^9/L with 18% blasts in the bone marrow. What phase of CML is she presenting with?

a) Chronic phase
b) Accelerated phase
c) Blast crisis
d) Asymptomatic disease
e) Remission of disease

A

B) Accelerated phase

A diagnosis of CML require a leucocytosis of 50-200x10^9/L and shows mature myeloid cells, basophils and neutrophils on a blood film.

Chronic phase - less than 5% blasts, can last up to 5 years, most diagnosed at this stage, 50% progress
Accelerated phase - 10-19% blasts, lasts up to 1 year
Blast crisis - over 20% blasts, resembles acute leukaemia but with a time frame of months

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19
Q

A 68 year old man goes to his GP for a routine check up. His bloods show a Hb of 10g/dL and a platelet count of 125x10^9/L. The GP also notices symmetrical painless lymphadenopathy. His temperature is 38.2C. She requests a blood film. What cells will she see?

A

This is CLL. 80% of cases are diagnosed on routine bloods. Patients present with BM failure, symmetrical painless lymphadenopathy and B Symptoms.

Bloods will show a lymphocytosis (5-300x10^9/L), a normocytic normochromic anaemia and low platelets. The blood film will show characteristic smear cells. Investigations can also include a bone marrow aspirate (lymphocytic replacement) and serum Ig, which will be low. There will be CD5+ve B cells.

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20
Q

What is Evan’s syndrome?

A

Refers to the association between CLL and autoimmunity e.g. AIHA and ITP.

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21
Q

What is Richter’s transformation?

A

Progression to diffuse large B cell lymphoma from CLL.

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22
Q

Which of these is associated with a bad prognosis in CLL?

a) 11q23 deletion
b) Hypermutation of Ig gene
c) CD38 -ve B cells
d) Decreased ZAP-70
e) 13q14 deletion

A

a) 11q23 deletion

Other associations with bad prognosis are CD38 +ve B cells and increased LDH.

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23
Q

Outline the management of CLL.

A
  1. Supportive - vaccines, prophylaxis
  2. Chemo - chloambucil, fludarabine, alemutzumab

If Richter’s - CHOP-R
17p deletion - BCR kinase inhibitor

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24
Q

Which of these is not a risk factor for lymphoma?

a) Chronic Helicobacter Pylori infection
b) Coeliac disease
c) HIV
d) CMV
e) EBV

A

d) CMV

Chronic antigen stimulation by a and b can cause specific types of lymphoma. The infections associated with lymphomas are HTLV-1 (adult T cell lymphoma or leukaemia), EBV (Burkitt’ or HL) and HIV (high grade B-NHL).

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25
Q

A 25 year old man presents to A&E after a heavy night out drinking. He complains of painful swelling in his neck over the past 3 hours. On questioning, he reports a 1 month history of nigh sweats, itching and fatigue. Investigation with a CT/PET scan confirms the presence of enlarged lymph nodes in the mediastinum and anterior chain of the neck. What is his diagnosis and what is the stage of his disease?

A

Hodgkin’s lymphoma. Stage 2B.

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26
Q

Which type of Hodgkin’s lymphoma shows:

Modified ‘popcorn’ Reed Sternburg cells

a) Nodular sclerosing
b) Mixed cellularity
c) Lymphocyte rich
d) Lymphocyte depleted
e) Nodular lymphocyte predominant

A

e) Nodular lymphocyte predominant

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27
Q

Which type of Hodgkin’s lymphoma shows:

Lacunar Reed Sternburg cells with lymph nodes in the neck and mediastinum.

a) Nodular sclerosing
b) Mixed cellularity
c) Lymphocyte rich
d) Lymphocyte depleted
e) Nodular lymphocyte predominant

A

A) Nodular sclerosing

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28
Q

Which type of Hodgkin’s lymphoma has:

The worst prognosis

a) Nodular sclerosing
b) Mixed cellularity
c) Lymphocyte rich
d) Lymphocyte depleted
e) Nodular lymphocyte predominant

A

D) Lymphocyte depleted

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29
Q

What are Reed Sternburg cells?

A

Bi or multinucleate ‘owl-eyed’ cells found in Hodgkin’s lymphoma

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30
Q

A 30year old man with a history of HL returns to see his oncologist after his GP noticed new enlarged neck lymph nodes on a recent check up. He has just finished a regimen of ABVD. What should be the next step in his management?

A

Autologous or allogenic SCT

Autologous - harvest stem cells from peripheral blood after giving G-CSF, freeze and then reintroduce after destruction of BM with chemotherapy.

Allogenic - transplant bone marrow from HLA matched donor to ‘empty BM’

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31
Q

Name the diagnosis:

Aggressive lymphoma associated with t(14;18) and BCL-6 expression which shows sheets of large lymphoid cells on histology.

a) Hodgkin’s lymphoma
b) Non- Hodgkin’s lymphoma
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

D) Diffuse large B cell lymphoma

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32
Q

Name the diagnosis:

Aggressive B cell lymphoma which shows cells with angular nuclei on histology and has a median survival time of 3-5 years.

a) Hodgkin’s lymphoma
b) Non- Hodgkin’s lymphoma
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

C) Mantle cell lymphoma

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33
Q

Name the diagnosis:

A type of lymphoma that often presents with continguous spread between lymph nodes and may present with a Pel-Ebstein cyclical fever.

a) Hodgkin’s lymphoma
b) EATL
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

A) Hodgkin’s lymphoma

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34
Q

Name the diagnosis:

May form from Richter’a transformation of CLL and also change into different types of lymphoma.

a) Hodgkin’s lymphoma
b) EATL
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

D) Diffuse large B cell lymphoma

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35
Q

Name the diagnosis:

Associated with t(11;14) and cyclin D1 dysregulation

a) Hodgkin’s lymphoma
b) EATL
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

c) Mantle cell lymphoma

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36
Q

Name the diagnosis:

Treated with watchful waiting and then a R-CVP regimen.

a) Hodgkin’s lymphoma
b) EATL
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

e) Follicular lymphoma

R-CVP: rituximab, cyclophosphamide, vincristine, prednisolone

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37
Q

Name the diagnosis:

A type of lymphoma that may resolve if the antigenic stimulus is removed

a) Hodgkin’s lymphoma
b) EATL
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

MALT - treatment of H.Pylori or Sjogren’s

38
Q

Name the diagnosis:

Shows a starry sky appearance on histology and presents with jaw involvement and abdominal masses.

a) Hodgkin’s lymphoma
b) EATL
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

Endemic Burkitt’s lymphoma - associated with EBV, found in Africa, young children (EMQ)

39
Q

Name the diagnosis:

Aggressive lymphoma associated with t(2;5) and Alk 1 protein. Large epitheloid lymphocytes on histology.

a) Hodgkin’s lymphoma
b) EATL
c) Mantle cell lymphoma
d) Diffuse large B cell lymphoma
e) Follicular lymphoma
f) MALT
g) Burkitt’s lymphoma
h) Peripheral T cell lymphoma
i) Adult T cell lymphoma
j) Anaplastic large cell lymphoma

A

j) Anaplastic large cell lymphoma - found in children and young adults

40
Q

A 55 year old woman presents to her GP with difficulty swallowing. She has a history of coeliac disease and has been a vegan for the past 5 years. On examination she has koilonychia, atrophic glossitis and angular chelosis. Her blood film shows hypochromic cells with some pencil cells. What is her diagnosis and the likely physiology?

A

Iron deficiency anaemia - reduced absorption/low dietary intake
This

41
Q

Five causes of iron deficiency anaemia and examples of each cause.

A

1) Blood loss e.g. GI loss secondary to Meckel’s diverticulum, ulcers, cancer, menorrhagia, hookworm
2) Increased use e.g. Pregnancy, lactation, growth
3) Decreased intake e.g. Diet, premature babies
4) Decreased absorption e.g. Coeliac
5) Intravascular haemolysis e.g. MAHA

42
Q

What is the pathophysiology of anaemia of chronic disease?

A

RBC productions is inhibited by cytokines e.g. Decreased erythropoietin receptor production by IFN, TNF and IL 1 and dysregulation of Fe metabolism due to increased hepcidin from the liver (Decreased transferrin and absorption leads to accumulation of iron in macrophages).

43
Q

Which of the following is not a cause of anaemia of chronic disease?

a) Malignancy
b) Chronic infection
c) Alcohol abuse
d) Vasculitis
e) Rheumatoid arthritis

A

c) Alcohol abuse - associated with both sideroblastic and myeloblastic anaemias but not ACD

44
Q

What is the pathophysiology of sideroblastic anaemia?

A

Ineffective erythropoiesis leads to iron loading and deposition in endocrine, liver and cardiac tissue (haemosiderosis)

45
Q

Bone marrow findings in sideroblastic anaemia

A

Ring sideroblasts - red blood cell precursors with iron in the mitochondria and a ring around the nucleus

46
Q

Which of the following is not a feature of megaloblastic anaemia?

a) Brisk reflexes
b) Irritability
c) Psychosis
d) Peripheral neuropathy
e) Paraesthesias

A

A) Brisk reflexes - megaloblastic anaemia causes an absent ankle reflex (LMN)

47
Q

What is the management of pernicious anaemia?

A

IM hydroxycobalamin (B12)

48
Q

Give 3 universal features of haemolytic anaemias in the blood.

A

Raised bilirubin, raised LDH, reticulocytosis

49
Q

Which of the following is not a precipitant of G6PD deficiency attacks?

a) Primaquine
b) Chronic infection
c) Sulfonamides
d) Moth balls
e) Broad beans

A

B) acute infection may lead to an attack. An attack will present as a rapid anaemia and jaundice due to haemolysis. All precipitants are oxidants which damage the red blood cells, which lack the protective glutathione.

50
Q

A 5 year old boy presents to A&E very lethargic with dark urine. 3 days ago he started taking primaquine as prophylaxis for an upcoming trip to Africa with his family. His blood film shows bite cells and Heinz bodies. What is his diagnosis and how is this diagnosed?

A

You would have a high suspicion of G6PD deficiency in this boy, which is endemic in malarial areas and can be precipitated by oxidants such as primaquine. Definitive diagnosis can only be achieved 2 or 3 months after the acute crisis when an enzyme assay is performed, as the unusual cells are destroyed in the haemolysis.

51
Q

A 5 day old baby presents to A&E with severe jaundice and splenomegaly. Phototherapy is ineffective and the BR level continues to rise. The paediatric haematology consultant believes that the child may have an enzyme deficiency. What is the inheritance pattern of this condition?

A

In a neonate with severe jaundice you would consider pyruvate kinase deficiency. This has an autosomal recessive pattern of inheritance.

52
Q

Describe the mutation associated with sickle cell anaemia.

A

Single base mutation of GAG to GTG (Glu to Val) in codon 6 of the beta chain of haemoglobin

53
Q

A teenager with a known autosomal recessive disease presents to his GP with impaired growth. He is anaemic, with a haemoglobin of 75g/l and has previously suffered from gallstones. What is the pathophysiology of this condition?

A

This teenager has sickle cell anaemia. A single base mutation in the beta chain of the haemoglobin molecule leads to defective B chain which causes red blood cells to sickle when there is reduced oxygen tension. Patients present with haemolysis (low Hb, splenomegaly, folate deficiency, aplastic crises) and vasoocclusion of the small blood vessels, eventually leading to CKD and retinopathy.

54
Q

Give 4 features of beta thalassemia

A

Skull bossing, maxillary hypertrophy, hairs on end skull X-ray, hepatosplenomegaly

55
Q

Describe the differences between warm and cold acquired haemolytic anaemias.

A

Warm - 37 degrees, associated with IgG, spherocytes on blood film, caused by lymphoma, CLL, SLE and methlydopa
Cold - under 37 degrees, IgM, associated with Raynaud’s, caused by EBV, Lyphoma, or mycoplasma

56
Q

A 4 year old girl who has recovered from a bout of chickenpox presents to A&E with SOB after playing outside in the snow. She is positive for Donath-Landsteiner antibodies. What is her diagnosis?

A

Paroxysmal cold haemoglobinuria - occurs after a viral infection. DL antibodies attach to red blood cells when cold and then in warming there is complement mediated haemolysis. Self-limiting condition.

57
Q

What is Ham’s test?

A

For paroxysmal nocturnal haemoglobinuria - acid induced lysis of abnormal red blood cells (no GPI RBC markers)

58
Q

How is eculizumab helpful in the management of paroxysmal nocturnal haemoglobinuria?

A

Prevents complement mediated haemolysis of RBCs by preventing binding

59
Q

An 8 year old girl presents to A&E acutely unwell with bloody diarrhoea. She went on a school trip to a petting farm yesterday. She looks pale and is struggling to keep food or water down. What is the likely diagnosis?

A

Haemolytic uraemia syndrome secondary to E.Coli - toxin damage to red blood cells leads to mechanical destruction of RBCs and they are pushed through a fibrin/platelet mesh (MAHA). This also leads to impaired renal function.

60
Q

Describe the features of Osler Weber Rendu syndrome.

A

Osler - like osler’s nodes = mucocutaneous lesions
Weber - like webs i.e. Telangiectasia
Rendu - like rendezvous i.e. where blood vessels meet in arterial-venous malformations
Plus connective tissue disease
Superficial bleeding into skin and mucosa as well as immediate bleeding post injury

61
Q

Compare the epidemiology of acute and chronic ITP.

A

Acute - mostly in children, 1:1 ratio F:M, associated with infection, platelet count less than 20000, self limiting course of 2-6 weeks

Chronic - mostly in adults, more common in F (3:1), not associated with infection, platelet count less than 50000, treat with IVIG and steroids

62
Q

A 7 year old boy presents with a swollen knee. His mother says that he fell over whilst playing football 3 days ago. The area is heavily bruised. There are no other sites of bleeding and you suspect haemophilia A. What distinguishes this condition from Von Willebrand’s disease?

A

Haemophilia A presents with prolonged bleeding after surgery or trauma whereas VW disease has mucocutaneous bleeding and may also have deep bleeding into muscles and tissues. In both, the APTT is prolonged, with a reduced factor 8 assay. In vWF bleeding time is also prolonged and vWF antigen is Decreased. VW disease affects platelet function as well as factor 8 production.

63
Q

What would investigations show in a case of DIC?

A

Low platelets, low fibrinogen, high FDP/D dimmer, long PT and APTT, long INR

64
Q

An 58 year old Afro-Caribbean lady presents to her GP complaining of fatigue. She has also noticed pain in her lower back and legs over the past couple of months with some lower leg swelling. Investigations show that she has hypercalcaemia, raised ESR and rouleaux on her blood film. A specific diagnostic protein is found in her urine. What is he pathophysiology of this disease?

A

Multiple myeloma - presents with CRAB: high calcium, renal impairment (which may cause nephrotic syndrome - swollen legs), anaemia (fatigue) and bone symptoms (bone pain, osteolytic lesions and fractures such as wedge compression of the spine). The protein found in the urine is Bence Jones protein.

MM is caused by a genomic instability secondary to infection or inflammation. The most common genetic changes are a 14q32 translocation or deletion in chromosome 13 leading to MGUS. Mutations include N-RAS, K-RAS and p16 methylation. There are bone marrow changes such as bone resorption and angiogenesis. Eventually, MGUS will progress to the symptomatic MM.

65
Q

What are the 2 possible features of cells in multiple myeloma on BM aspirate?

A

Mature plasmacytic - see clumped chromatin and increased cytoplasm
Immature plasmablastic - prominent nucleoli with reticular chromatin

66
Q

Name 3 drugs that may be used in the management of multiple myeloma.

A

Supportive therapy with a bisphosphonate e.g. alendronate
Chemotherapy with melphalan or a proteasome inhibitor such as bortezomib
Steroids such as dexamethasone or prednisolone

67
Q

Difference in the diagnostic criteria for MGUS and smouldering myeloma.

A

MGUS - less than 10% plasma cells in bone marrow and less than 30g/l monoclonal Ig but 1-2% progress to MM per year.

Smouldering myeloma - over 10% plasma cells in bone marrow but no symptoms (CRAB) or organ involvement.

68
Q

A confused 73 year old man is brought to the GP by his daughter. She is worried that over the past 6 months he has become increasingly weak and fatigued and is now unable to take care of himself. A bone marrow aspirate shows lymphoplasmacytoid cells along with a high serum IgM. What is his diagnosis and name 1 possible treatment option.

A

Waldenstrom’s Macroglobulinaemia (aka lymphoplasmacytoid lymphoma). This is a low grade non Hodgkin’s lymphoma with a lymphoplasmacytoid cell infiltration of the lymph nodes and bone marrow. It presents with B symptoms and hyperviscosity (visual problems, confusion, congestive HF, muscle weakness).

Management - plasmapheresis, chlorambucil, cyclophosphamide

69
Q

Outline the common pathophysiology of myelodysplastic syndromes.

A

They are progressive acquired haematopoietic disorders with ineffective proliferation and abnormal mature myeloid cells.

Therefore there are functionally defective cells combined with a numerical reduction.

70
Q

Which of the following is an abnormality found on investigation for myelodysplastic syndrome?

A) Hypegranulated WBCs
B) Rouleaux
C) Shortening of intrasegment neutrophil nucleus fragments
D) Pelger Huet anomaly (bilobed neutrophils)
E) Dacrocytes

A

D) Pelger Huet anomaly

Also seen are hypercellular bone marrow, ring sideroblasts, hypogranulated WBCs, dysplastic megakaryocytes and myelokathexis I.e. Lengthening of intrasegment neutrophil nucleus filaments

71
Q

What are the three main categories of myelodysplastic syndrome?

A
  1. Refractory anaemia
    - with ring sideroblasts
    - excess blasts I (5-9%)
    - excess blasts II (10-19%)
  2. Refractory cytopaenia with multi lineage dysplasia
  3. Unclassified - neutropenia and thrombocytopenia
72
Q

What are the main causes of death from myelodysplastic syndrome?

A

1/3 infection, 1/3 bleeding, 1/3 leukaemia (transformation to AML for 50% within 1 year)

73
Q

Match the presentation to the type of aplastic anaemia:

A short 40 year old woman with multiple endocri e problems, hepatic impairment and skeletal abnormalities is newly diagnosed with AML.

a) Fanconi anaemia
b) Dyskeratosis congenita
c) Schwachman Diamond Syndrome
d) Diamond Blackfan syndrome
e) Idiopathic

A

c) Schwachman Diamond Syndrome - recessive disorder causing a neutrophilia

74
Q

Match the presentation to the type of aplastic anaemia:

A patient with increased skin pigmentation, oral leukoplakia, nail dystrophy and bone marrow failure.

a) Fanconi anaemia
b) Dyskeratosis congenita
c) Schwachman Diamond Syndrome
d) Diamond Blackfan syndrome
e) Idiopathic

A

B) dyskeratosis congenita - x-linked with chromosomal instability and telomere shortening

75
Q

Match the presentation to the type of aplastic anaemia:

Cytopaenia and hypocellular bone marrow in a patient with a long history of SLE.

a) Fanconi anaemia
b) Dyskeratosis congenita
c) Schwachman Diamond Syndrome
d) Diamond Blackfan syndrome
e) Secondary

A

e) secondary (immune attack on bone marrow)

76
Q

Match the presentation to the type of aplastic anaemia:

A 7 year old boy with a pancytopaenia, renal malformation, microopthalmia, short stature, skeletal abnormalities and skin pigmentation.

a) Fanconi anaemia
b) Dyskeratosis congenita
c) Schwachman Diamond Syndrome
d) Diamond Blackfan syndrome
e) Idiopathic

A

A) Fanconi anaemia - autosomal recessive

77
Q

Match the presentation to the type of aplastic anaemia:

A 3 month old baby with an anaemia and dysmorphic red cells but normal WCC and platelets.

a) Fanconi anaemia
b) Dyskeratosis congenita
c) Schwachman Diamond Syndrome
d) Diamond Blackfan syndrome
e) Idiopathic

A

d) Diamond Blackfan Syndrome

78
Q

What are the Comitta criteria for aplastic anaemia?

A

Reticulocytes less than 1%
Neutrophils less than 0.5 x 10^9
Platelets < 20 x 10^9

Severe requires 2 of 3 criteria above.

79
Q

Which of the following is an indication for a red blood cell transfusion?

a) 20% blood volume loss
b) An Hb of 78g/l in a critical care patient
c) An Hb of 75g/l in a cancer patient undergoing chemotherapy
d) A young male with a blood pressure of 105/65
e) A young woman with iron deficiency anaemia but no symptoms

A

C) Hb < 80g/l in a chemotherapy patient is an indication for transfusion

Hb < 70g/l in a critical care patient
> 30% blood loss
Symptomatic anaemia

80
Q

Give 2 indications for giving a patient FFP.

A

Indications

  • blood loss > 150ml/min
  • DIC with bleeding
  • PT ratio over 1.5x normal
81
Q

What is the blood transfusion reaction demonstrated in the case?

A 30 year old woman who had a blood transfusion 8 days ago after severe post partum haemorrhage presents to A&E with increasing jaundice. Her U&Es show signs of acute kidney failure and her full blood count shows she is anaemic. Reticulocytes are seen on a blood film.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

h) Delayed haemolytic transfusion reaction

The history of increasing jaundice with anaemia clearly points to a haemolytic cause in this case. Delayed haemolysis usually occurs 5-10 days after transfusion. Previous sensitisation to antigens causes the production of IgG on repeated exposure.

Ix show increased bilirubin, Decreased Hb, increased reticulocytes and deranged U&Es.

82
Q

What is the blood transfusion reaction demonstrated in the case?

An 8 year old boy receives a plasma transfusion after being in an RTA. Soon after the transfusion is started the boy develops a wheeze and begins to be short of breath.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

e) Anaphylaxis

The rapid nature of this boy’s deterioration after the transfusion is started and his symptoms of wheeze and SOB are strongly indicative of anaphylaxis. This occurs due to IgE antibodies and mast cell degranulation.

83
Q

What is the blood transfusion reaction demonstrated in the case?

A 25 year old man undergoing a transfusion has a sudden increase in temperature from 37.5 to 38.5 and begins to suffer from chills and rigors.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

a) Febrile non-haemolytic transfusion reaction

Presents as seen above.
Can be caused by stored WBCs releasing cytokines which then cause a reaction when transfused into the patient.
Mx - stop or slow transfusion and give paracetamol

84
Q

What is the blood transfusion reaction demonstrated in the case?

A 70 year old man who has a transfusion after previously being given a bag of saline presents with increasing shortness of breath and decreasing oxygen saturations. His heart rate and BP are both elevated. A CXR shows pulmonary oedema.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

f) Transfusion associated circulatory overload

It is likely that this older gentleman has some renal impairment which is preventing homeostasis of fluid balance. This may also happen in heart failure or patients with a low albumin.

85
Q

What is the blood transfusion reaction demonstrated in the case?

A 5 year old child who has regular blood transfusions for beta thalassemia presents to A&E with a new onset purpuric rash. His last blood transfusion was a week ago.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

l) Post transfusion purpura

Occurs in patients immunised to HPA-
1a who therefore have antibodies to certain blood products. The purpura develops 7-10 days after transfusion. It may self resolve or be life threatening.

Mx - IVIG infusion

86
Q

What is the blood transfusion reaction demonstrated in the case?

A 35 year old man with HIV presents with severe diarrhoea after a blood transfusion. His liver enzymes are raised, his Hb is low and his platelet count is also low.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

k) Transfusion associated GVHD

Donor lymphocytes that are not destroyed by the patient’s immune system (e.g. In immunosuppression) attack the patient’s tissue HLA in the gut, liver, skin, BM etc. Therefore presents with diarrhoea, liver failure, skin desquamation and BM failure.

87
Q

What is the blood transfusion reaction demonstrated in the case?

A 30 year old woman complains of chest pain 30 mins after a blood transfusion has started. She collapses on the post natal ward and the nurses bleep the doctors on call. Her obs show a fever of 39 degrees with a BP of 95/60 and a HR of 110. She is flushed and when she is roused she begins to vomit.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

c) Acute intravascular haemolysis (wrong blood)

This woman has been given someone else’s blood! There is rapid haemolysis which causes haemoglobinuria as well as all of the above symptoms. It is an IgM mediated reaction.

Ix - FBC, biochemistry, repeat crossmatch, DAT

88
Q

What is the blood transfusion reaction demonstrated in the case?

A 5 year girl who is on chemotherapy is given platelets after it is found that her platelets are 10 x 10^9/L. The nurses who gave the platelets was called away to another patient after removing the bag from the fridge and does not know how long they were out for. The girl has a fever of 38.5 and looks miserable.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

d) Bacterial contamination

Secondary to endotoxins. More common reaction in platelets, especially if left out of fridge for more than 30 mins.

89
Q

What is the blood transfusion reaction demonstrated in the case?

A 33 year old man who has a transfusion after an RTA presents with shortness of breath, Decreased oxygen saturations and tachycardia 5h after he was admitted to hospital. A CXR shows bilateral patchy infiltrates.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

g) TRALI

This is caused by anti WBC Abs transfused in the donor blood which enter the recipient’s circulation and caused WBC aggregates. These lodge in pulmonary capillaries and lead to activation of neutrophils, which produce proteolytic enzyme and oxygen radicals, causing diffuse lung damage.

The patient will have the symptoms described above.

90
Q

What is the blood transfusion reaction demonstrated in the case?

A 10 year old child who has regular blood transfusions for beta thalassemia presents to A&E with fatigue, hyperpigmentation and joint pain. On examination he has hepatosplenomegaly.

a) Febrile non-haemolytic transfusion reaction
b) Allergic transfusion reaction
c) Acute intravascular haemolysis
d) Bacterial contamination
e) Anaphylaxis
f) Transfusion associated circulatory overload
g) Transfusion related acute lung injury
h) Delayed haemolytic transfusion reaction
i) Infection
k) Transfusion associated GVHD
l) Post transfusion purpura
m) Iron overload

A

m) Iron overload

Build up of Fe in organs after regular transfusions.

Mx - desferrioxamine if ferritin over 1000

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