Haematology pathology

Question 1

15593 – Neutropenia may be associated with
1: cotrimoxazole therapy
2: systemic lupus erythematosus
3: polyarteritis
4: Addisonian (pernicious) anaemia

Answer 1

TTFT
Refer to Robbins, 6th Ed, Ch 15, page 646-647

Question 2

13077 – Neutropenia may be associated with
1: thiouracil therapy
2: systemic lupus erythematosus
3: polyarteritis
4: Addisonian (pernicious) anaemia

Answer 2

TTFT
Patients with systemic lupus erythematosus have leukocyte autoantibodies causing neutropenia (B true). Agranulocytoses is encountered as an idiosyncratic reaction to a number of drugs including thouracil (A true) while the white cell share in the general reduction in cell cycling consequent on vitamin B12 deficiency (D true). Leukocytosis characterises the reaction in polyarteritis (C false).

Question 3

24324 – Microcytic hypochromic anaemia
1: is due to iron deficiency
2: is common in females of menstrual age
3: may be associated with a lack of vitamin B6 (pyridoxine)
4: is a common complication of gastrectomy

Answer 3

TTFT
Robbins 6th ed. Pages: 449; 623; 629 Ch 14, p627-630

Question 4

23754 – A marked increase in blood eosinophils often accompanies
1: Trichinella spiralis
2: Loeffler’s syndrome
3: systemic vasculitis
4: acute renal allograft rejection

Answer 4

TTTF
Robbins 6th ed. Pages: 648; 738 Roitt 9th ed. Pages: 277; 357

Question 5

13082 – A marked increase in blood eosinophils often accompanies
1: hydatid disease
2: Loeffler’s syndrome
3: Delayed type hypersensitivity
4: Polyarteritis nodosa

Answer 5

TTFT
Eosinophilia characteristically accompanies a number of parasitic diseases, including hydatid disease (A true). It is a frequent concomitant of polyarteritis nodosa (D true) and Loeffler’s syndrome (B true), a transient condition associated with helminth infestation. It is not a feature of delayed hypersensitivity (C false).

Question 6

13120 – Conditions predisposing to thrombosis include
1: polyarteritis nodosa
2: giant cell arteritis
3: Buerger’s disease
4: Takayasu’s disease

Answer 6

TTTT
Thrombosis is very likely in those diseases of the vessels in which the intima is inflamed. All four of the conditions listed show intimal inflammation (A,B,C,D true).

Question 7

22764 – Antithrombin III
1: potentiates the action of PGI\b2 (prostacyclin)
2: deficiency causes recurrent phlebothrombosis
3: antagonises the actions of a wide spectrum of activated serum proteases
4: deficiency may be successfully treated with low dose heparin

Answer 7

FTTT
Robbins 5th ed. Pages: 100-101

Question 8

15800 – Factors which tend to localise and limit thrombocoagulation (pathological or in response to injury) include
1: clearance of activated prothrombocoagulants
2: local degradation of clotting factors
3: local arterial/arteriolar constriction
4: secretion of protease inhibitors by adjacent intact endothelium

Answer 8

TTFT
This is the reinstatement of homeostatic mechanisms by the intact endothelium. Without these feedback mechanisms, any initiation of the protective or pathological thrombocoagulant state would progress inexorably.

Question 9

23334 – Acute intravascular haemolysis, due to incompatible blood transfusion causes
1: decreased plasma haptoglobin level
2: methaemalbuminaemia
3: splenomegaly
4: obstructive hyperbilirubinaemia

Answer 9

TTFF
Robbins 5th ed. Chapter: 13 Pages: 587-588

Question 10

21923, 13067 – Acute intravascular haemolysis is characteristically accompanied by
1: reduced level of serum haptoglobin
2: raised plasma haemoglobin
3: haemoglobinuria
4: methaemalbuminaemia

Answer 10

TTTT
Robbins 6th ed. CHAPTER: 14 PAGE: 606. Following acute intravascular haemolysis the binding power of the plasma (due to haptoglobin, haemopexin and albumin) is soon exhausted. The level of serum haptoglobin is thus reduced (A true). Haemoglobinuria soon follows, along with
methaemalbuminaemia (C and D true). Following acute intravascular haemolysis the plasma haemoglobin increases (B true).

Question 11

13105 – Disseminated intravascular coagulation may cause the development of
1: haemolytic anaemia
2: renal failure
3: haemorrhagic diathesis
4: circulating anticoagulant substances

Answer 11

TTTT
The extensive intravascular coagulation associated with disseminated intra-vascular coagulation (DIC) consumes coagulation factors, and this is further exacerbated by the action of the fibrinolytic system. Thus DIC is associated with a haemorrhagic diathesis (C true). The deposition of fibrin within the microvasculature may lead to haemolytic anaemia (A true). Fibrinogen breakdown products include anticoagulant substances (D true). The events characterising DIC result in renal ischaemia, even to a degree of bilateral renal cortical necrosis, as can a number of the conditions which precipitate DIC (B true). A number of the conditions which may cause DIC, can themselves cause renal failure of other mechanisms.

Question 12

15805 – Disseminated intravascular coagulation (thrombocoagulation) may cause
1: acute oliguric renal failure
2: haemorrhage
3: neutropenia
4: diffuse alveolar damage (adult respiratory distress syndrome)

Answer 12

TTFT
Responses 1 and 4 relate to the likelihood of thrombo-occlusive problems occurring as a result of formation of platelet-fibrin masses in the blood stream with resulting emboli in renal and/or pulmonary microcirculation. The spontaneous haemorrhage relates to the ‘consumptive’ component of DIC with consequent lack of platelets and coagulation proteins in the blood. WBC are not involved in the
‘consumption’.

Question 13

23329 – Severe intravascular haemolysis may be seen as a complication of
1: clostridial infections
2: hereditary spherocytosis
3: lead poisoning
4: disseminated intravascular coagulation (thrombocoagulation)

Answer 13

TFFT
Robbins 5th ed. Chapter: 13 Pages: 587-588

Question 14

22093 – Intravascular haemolysis may occur as a result of
1: alpha-thalassemia
2: Clostridium perfringens infection
3: mechanical heart valves
4: incompatible blood transfusion

Answer 14

FTTT
Robbins 5th ed. Chapter: 13 Pages: 586-587; 506-600

Question 15

24184 – In Australia and New Zealand, donor blood for transfusion is screen-tested for markers of the following viruses
1: cytomegalovirus (CMV)
2: hepatitis B and C (HBV and HCV)
3: human immunodeficiency viruses (HIV 1/2)
4: Epstein-Barr virus (EBV)

Answer 15

FTTF
Blood Transfusion & Component Therapy ARC 1994 Page 3

Question 16

15207 – For transfusion purposes, stored whole blood can be considered to
be a suitable clinical source of
1: oxygen-transportable haemoglobin
2: functional platelets
3: coagulation factors V and VIII
4: functional granulocytes

Answer 16

TFFF
Refer to Australian Red Cross NTBC Booklet, Jan 1994, supplement Feb 1998

Question 17

7284 – In a patient undergoing massive transfusion, severe capillary-type oozing develops
1: blood for haemoglobin, platelet count, INR and APTT should be taken immediately
2: transfusion of platelets and fresh frozen plasma should await the assessment of the laboratory results
3: fresh frozen plasma is indicated even if INR and APTT are normal
4: if INR and / or APTT are prolonged, estimation of fibrinogen is important
5: fibrinogen deficiency may be corrected by administration of cryoprecipitate
6: capillary oozing, especially if accompanied by a falling blood pressure, may be a sign of a haemolytic transfusion reaction

Answer 17

TFFTTT

Question 18

20271 – S. The ABO blood group system is the most important in transfusion practice BECAUSE R. Anti-A and Anti-B antibodies are regularly found in subjects whose red cells lack the corresponding antigen

Answer 18

S is true, R is true and a valid explanation of S
Robbins 6th ed. PAGE: 473 Blood Transfusion Therapy P40

Question 19

7278 – Each of the following statements may be True or False
1: Hepatitis G is an important source of hepatitis infection in blood transfusion practice
2: febrile non-haemolytic reactions to donor white cells occur in 1% of all transfusions
3: autologous blood transfusion eliminates the infective risks of blood transfusion
4: urticaria during blood transfusion is usually a reaction to donor white cells
5: neutrophils have a half-life in the blood of 3-4 days

Answer 19

FTFFF

Question 20

13062 – One week after a severe haemorrhage, the blood is likely to show an increased number of
1: reticulocytes
2: acanthocytes
3: polychromatic erythrocytes
4: larger than normal red cells

Answer 20

TFTT
One week after acute haemorrhage the bone marrow shows normoblastic hyperplasia, and this is reflected in an increase in the number of reticulocytes (immature red cells) in the circulation (A true). These are larger than normal red cells (D true) and often exhibit polychromatic staining (C true). Acanthocytes are characteristic of haemolytic anaemia. They are not a feature of the blood picture following a severe haemorrhage (B false).

Question 21

21803 – One week after a haemorrhage of about one litre, the peripheral blood will usually show greatly increased numbers of
1: immature white blood cells
2: platelets
3: polychromatic erythrocytes
4: lymphocytes

Answer 21

FFTF
Robbins 5th ed. Chapter: 13 Page: 587

Question 22

25427 – Bone marrow grafting in humans
1: may be carried out using cord blood as a source of haemo- poietic stem cells
2: has improved primary engraftment if cyclosporin A is used for immunosuppression
3: requires matching of only HLA Class I antigens to avoid graft versus host disease
4: has improved survival if donor B lymphocytes are removed before grafting

Answer 22

TTFF
Roitt Essential Immunology 8th ed. Pages: 354-355

Question 23

13100 – Cells likely to be seen in the peripheral blood of a patient whose bone marrow has been extensively replaced, as in myelofibrosis, include
1: normoblasts
2: megaloblasts
3: myelocytes
4: myeloblasts

Answer 23

TFTT
The patient whose bone marrow is replaced can compensate by producing bone marrow in other sites, eg in the spleen. Haemopoiesis in extramedullary sites lacks the regulatory mechanisms operating in the bone marrow, and primitive cells are apt to enter the circulation. Thus normoblasts, myelocytes and myeloblasts are seen in the peripheral blood (A,C and D true). The deficiencies which lead to megaloblastic differentiation do not usually complicate the picture in intramedullary haemopoiesis (B false).

Question 24

9770 – Amyloid associated protein (AA) is greatly elevated in serum in
1: multiple myeloma
2: Crohn’s disease
3: patients on long term haemodialysis
4: rheumatoid arthritis

Answer 24

FTFT
Robbins, 6th ed, Ch 7

Question 25

23704 – Systemic amyloidosis is commonly associated with
1: bleeding
2: proteinuria
3: neutrophil leucocytosis
4: parenchymal atrophy of affected organs

Answer 25

TTFT
Robbins 5th ed. Page: 231-8 1312

Question 26

17748 – Likelihood of infections in advanced, untreated myeloma is contributed to by
1: reduced levels of normal (non-myeloma) immunoglobulins
2: high rate of infection by low-grade (‘opportunistic’) pathogens
3: depression of cellular (T cell) immunity
4: progressive, unrelenting viral infections

Answer 26

TFFF
The raised total level of plasma immunoglobulins is predominantly due to the monoclonal tumour-manufactured Ig (which is, protectively speaking, ‘nonsense Ig’). The infections which pose the threat to life are, therefore, the ‘usual’ pyogenic infections for which adequate protection is lost. T-cell immunity is well conserved and so the relentlessly progressive viral infections which are characteristic of depressed T-cell function are not a feature of myeloma.

Question 27

15538 – Regarding multiple myeloma
1: the antibodies produced by a given myeloma are likely to have the same heavy chains
2: IgG is often excreted in the urine
3: parts of antibody molecules may be produced rather than whole molecules
4: the majority of myelomas produce IgM antibodies

Answer 27

TFTF
Refer to Robbins, 6th Ed, page 663-664

Question 28

20007 – Patients with multiple myeloma commonly show all the following EXCEPT
A. increased susceptibility to pyogenic infections
B. bone fracture
C. renal failure
D. normochromic, normocytic anaemia
E. peripheral blood plasmacytosis

Answer 28

E
Robbins 5th ed. CHAPTER: 14 PAGE: 664-665

Question 29

17823 – The commonest cause of death in multiple myeloma is
1: renal failure
2: amyloid-related multiple organ failure
3: intestinal infarction due to hyperviscosity syndrome
4: infections by pyogenic bacteria
5: cardiac arrhythmia due to hypercalcaemia

Answer 29

FFFTF
What’s to add? The specific immunoglobulin lack (not only the lack of ability to make ‘new’ immunoglobulins to combat infections with ‘new’ invaders, but loss of adequate levels from previous
infections) leads to particular susceptibility to infections with encapsulated bacteria (eg pneumococci). All of the others are hazards for patients with multiple myeloma, but pyogenic infections are the
commonest killer.

Question 30

14843 – Symptomatic haemophilia A (factor VIII deficiency)
1: commonly causes severe `spontaneous’ bleeding when plasma levels fall to approximately 25% of normal
2: characteristically causes petechial and ecchymotic haemorrhages
3: does not occur in females
4: requires assay of plasma factor VIII levels for reliable diagnosis

Answer 30

FFFT
Refer to Robbins, 6th Ed, Ch 14, page 638-639

Question 31

12944 – A previously normal adult suffers a ruptured spleen in an automobile accident. Removal of his spleen causes
1: transient thrombocytosis
2: increase in red cell survival time
3: an increased liability to infection
4: reduced iron transport in blood

Answer 31

TFTF
In the immediate postoperative period following splenectomy, the platelet count usually rises to 600-1000 x 109/1 in the first 7-10 days. This is usually transitory with a fall to near normal values within 1-
2 months (A true). Although a reticulocytosis often occurs, red cell survival time will not be altered (B false). Overwhelming infection is an uncommon but serious complication following splenectomy (C true). Changes in iron metabolism are not seen following splenectomy (D false).

Question 32

23004 – Tumour cells disseminated by the blood stream
1: have enhanced implantability when aggregated with platelets
2: are destroyed by cytotoxic T lymphocytes
3: are distributed by affinity with endothelial cell receptors
4: have a limited chance of survival

Answer 32

TFTT
Robbins 6th ed. PAGE: 305. Pending review. Jan 2003

Question 33

15768 – The following haematological problems commonly occur either as a direct effect or as a complicating result of chronic alcohol abuse
1: coagulation disorder
2: microcytic, hypochromic anaemia
3: normocytic, normochromic anaemia
4: erythrocyte macrocytosis

Answer 33

TTTT
Coagulation disorders of potential disaster magnitude may be asymptomatic in cirrhotic patients with occult liver failure, as may be oesophageal varices (or alcohol-related peptic ulcer) of sufficient severity to cause chronic bleeding with iron deficiency (response 2) or occult more acute recent bleeding (response 3). Macrocytosis is a common marker of alcohol abuse with subclinical, asymptomatic folate deficiency.

Question 34

24144 – A high percentage saturation of transferrin with iron is present in
1: transfusion haemosiderosis
2: haemochromatosis
3: blacks consuming food and beverages prepared in iron utensils
4: polycythaemia vera

Answer 34

TTTF
Robbins 5th ed. PAGE: 73; 28; 610-616; 862

Question 35

25474 – Concerning malignant lymphoma
1: the majority of non-Hodgkin lymphomas are of B-cell origin
2: they can easily be differentiated from anaplastic carcinoma by routine H & E staining
3: in Burkitt’s lymphoma the c-myc oncogene is commonly suppressed
4: it is 35 times more common in transplanted patients than in normals

Answer 35

TFFT
Roitt Essential Immunology 9th ed. Page: 387-390

Question 36

15202 – A lymph node biopsied from a patient diagnosed as showing malignant lymphoma (non-Hodgkin’s type)
1: is likely to contain Reed-Sternberg cells
2: is more likely to be composed of malignant T cells than B cells
3: will not contain reactive lymphocytes of the same lineage (ie T or B) as the neoplastic cells
4: will contain a monoclonal population of tumour cells

Answer 36

FFFT
Refer to Robbins, 6th Ed, Ch 15, page 652-653

Question 37

13052, 22454 – An increased incidence of lymphoma and/or leukemia is associated with
1: treatment with alkylating agents
2: human immunodeficiency virus (HIV) infection
3: hereditary immunological deficiency syndromes
4: autoimmune haemolytic anaemia

Answer 37

TTTF
Robbins 6th ed. Chapters: 6; 7 Pages: 234; 247; 309.
Alkylating agents are direct-acting carcinogens with particular reference to leukaemias and lymphomas (A true); HIV infection is similar as are other (hereditary, but also therapeutically-induced) immune deficiency syndromes (B and C true). Autoimmune haemolytic anaemia has no precancerous connotations (D false).

Question 38

21928 – Which of the following drugs may cause red blood cells deficient in glucose-6-phosphate dehydrogenase to undergo lysis?
1: primaquine
2: probenecid
3: aspirin
4: sulphonamides

Answer 38

TTTT
Robbins 6th ed. Page: 610

Question 39

12650 – S: Haemophiliacs usually have a normal ‘bleeding time’ test because R: haemophiliacs have a normal platelet aggregatory response to microvascular injury

Answer 39

S is true, R is true and a valid explanation of S
Patients with haemophilia have normal amounts of functional von Willebrand factor (vWF). The skin bleeding time is prolonged when vWF is reduced or dysfunctional but is normal when the only
coagulation abnormality is reduction of Factor VIII. Thus the statement and response are both true with the response being a valid explanation of the statement.

Question 40

12680 – S: Patients suffering from von Willebrand’s disease bleed excessively post operatively because R: in von Willebrand’s disease there is usually a deficiency of Factor VIII and a platelet defect

Answer 40

S is true, R is true and a valid explanation of S
In von Willebrand’s disease there is an inherited deficiency of the named factor (vWF) which is necessary for platelet adhesion, serving as a molecular bridge between platelets and collagen. This bridge withstands high shear forces generated by flowing blood. VWF is the predominant moiety of a VIII-vWF complex, serving as a carrier for factor VIII. A deficiency of vWF gives rise to a secondary decrease in factor VIII level.

Question 41

14940 – S: Patients with Factor IX deficiency are very rarely asymptomatic because R: it is inherited as an X-linked recessive disorder

Answer 41

S is false and R is true
Refer to Robbins, 6th Ed, Ch 14, page 639

Question 42

12548, 19492 – A favourable response to splenectomy is most likely to occur in
A. hereditary elliptocytosis
B. thalassaemia major
C. paroxysmal nocturnal haemoglobinuria
D. hereditary spherocytosis
E. autoimmune haemolytic anaemia

Answer 42

D
Walter & Israel 6th Ed. CHAPTER: 52 PAGE: 647-650.
Splenectomy is of no value in the haemoglobinopathies or in paroxysmal nocturnal haemoglobinuria. It has some value in acquired haemolytic anaemia and hereditary elliptocytosis. The indication par excellence for splenectomy is hereditary spherocytosis.

Question 43

7303 – A patient is bleeding excessively post-operatively. INR, APTT and platelet count are normal
1: two units of fresh frozen plasma should be given
2: despite the normal APTT, the patient may have haemophilia A or von Willebrand disorder
3: chronic renal failure is not likely to be a contributing factor
4: a normal platelet count excludes platelet dysfunction as a possible cause
5: if the cause is due to reduced platelet function, an infusion of desmopressin may be helpful

Answer 43

FTFFT