Haematology Flashcards

1
Q

Which is a cause of platelet dysfunction? (August 2014)

a. Splenomegaly
b. Uraemia

A

ANSWER: Uraemia

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2
Q

Which is the most likely to present as petechial mucosal haemorrhages? (March 2015)

a. Christmas disease (Haemophilia B)
b. Lupus anticoagulant
c. Vitamin K deficiency

A

Haemophilia B presentation:
o Bleeding during the neonatal period
o Large haematoma as a toddler following minor trauma

Lupus anticoagulant presentation:
o Vascular thrombosis

Vitamin K deficiency:
o Symptoms in the presence of hypoprothrombinaemia
o Mucosal and subcutaneous bleeding
- Epistaxis, Haematoma, GI haemorrhage, Menorrhagia, Gum bleeding, Haematuria, Oozing from venupuncture sites
o On examination will see bruises, petechiae, haematomas and oozing

ANSWER: Vitamin K deficiency

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3
Q

Flow cytometry is used for: (September 2013)

a. Determining the clonality of lymphomas
b. Detecting microsatellite instability in colon cancer
c. Determining HER-2 status in breast cancer

A

ANSWER: Determining the clonality of lymphomas

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4
Q

Man with HIV presenting with multiple small lesions in the liver and spleen. Most likely?

a. Candida
b. Lymphoma metastases

A

Hepatosplenic candidiasis
o Multiple small hypodense lesions in the liver and spleen
o Immunosuppressed patients (e.g. HIV, chemotherapy)

ANSWER: Candida

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5
Q

What is the most common cause of miliary nodules in the spleen? (March 2014)

a. Cardiac failure
b. Myelofibrosis
c. CLL
d. Portal hypertension
e. Polycythaemia vera

A
Differential diagnosis of splenic siderotic nodules:
o	Portal hypertension (intrahepatic cause or splenic vein thrombosis)
o	Paroxysmal nocturnal haemoglobinuria
o	Sickle cell anaemia
o	Leukaemia
o	Lymphoma
o	Patients receiving blood transfusions
o	Acquired haemochromatosis
o	Splenic haemangiomatosis
o	Splenic neoplasm:
            - Littoral cell angioma of the spleen, Haemangioendothelioma of the spleen, Angiosarcoma of the spleen
o	Cardiac myxomas
o	Hepatosplenic schistosomiasis

ANSWER: Presuming the question is referring to siderotic nodules, the most likely answer is portal hypertension

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6
Q

Splenectomy increases susceptibility to which bacteria? (August 2014)

a. H. influenzae
b. Tuberculosis
c. Mycosis fungoides

A
Splenectomy increases susceptibility to encapsulated bacteria. These include:
o	H. influenzae
o	Pneumococcus
o	Meningococcus
o	GBS
o	Klebsiella
o	Salmonella typhi

ANSWER: Splenectomy increases susceptibility to H. influenzae

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7
Q

Which is the least likely cause of moderate to severe splenomegaly? (March 2016)

a. Schistosoma mansoni
b. Burkitt lymphoma
c. Polycythaemia vera
d. Myelofibrosis
e. Sickle cell anaemia

A
Causes of massive splenomegaly:
o Haematological:
	Leukaemia (especially CML)
	Polycythaemia vera
	Myelofibrosis
o Infection:
	Malaria
	Schistosoma mansoni causes splenomegaly by causing portal hypertension (periportal fibrosis)

Sickle cell - commonly enlarged in first decade, may persist or atrophy and undergo autosplenectomy

ANSWER: Burkitt lymphoma is least likely to cause moderate to severe splenomegaly

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8
Q

Which does not cause splenomegaly? (March 2017)

a. Biliary ascariasis
b. Schistosomiasis
c. Malaria

A

ANSWER: Biliary ascariasis

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9
Q

Which is least likely to result in splenomegaly? (March 2017)

a. Hepatitis B
b. Budd Chiari

A

Disorders associated with splenomegaly:
o Infections:
Non-specific splenitis, EBV, TB, Typhoid fever, Brucellosis, CMV, Syphillis, Malaria, Histoplasmosis, Toxoplasmosis, Schistosomiasis, Echinococcus

o Congestive states related to hypertension:
Cirrhosis, Portal or splenic vein thrombosis, Cardiac failure

o Lymphohaematogenous disease:
HL, NHL and lymphocytic leukaemia - Excluding Burkitt lymphoma, MM, Myeloproliferative disorders, Haemolytic anaemia, Thrombocytopaenia purpura

o Immunologic inflammatory conditions:
RA and SLE

o Storage disorders:
Gaucher, Niemann-Pick disease, Mucopolysaccaridoses

o Miscellaenous:
Amyloidosis, Primary neoplasms and cysts, Secondary neoplasms

Budd-Chiari is a cause of acute splenomegaly

ANSWER: Hepatitis B and Budd-Chiari could both be a cause of splenomegaly provided there is resultant cirrhosis or hepatic congestion

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10
Q

Splenic infarcts are most commonly from: (March 2015)

a. Sickle cell
b. Spherocytosis
c. Thalassaemia

A

Aetiology of splenic infarction:
o Haematological disorders (younger patients)
Sickle cell

o Embolic events (older patients)
Infective endocarditis
Non bacterial endocarditis (marantic endocarditis)

o	Others:
	Splenic vascular disease and aneurysms
	Variant anatomy +/- torsion
	Collagen vascular disease
	Pancreatitis
	Non-haematological malignancies
	Trauma

ANSWER: Splenic infarcts are most commonly from sickle cell disease

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11
Q

Which is least associated with Sickle cell disease? (March 2014)

a. Acute chest pain
b. Shock with splenic sequestration
c. Aplastic crisis
d. Staph aureus with autosplenectomy

A

Clinical manifestations of Sickle cell disease:
o Bone pain: infarction, OM
o Pulmonary: ACS, recurrent pneumonia (functional asplenia), chronic lung disease
o Abdominal: vaso-occlusive crises, sequestration syndrome (Rapid pooling of blood in the spleen leading to intravascular volume depletion)
o Haemolytic anaemia
o Autosplenectomy: impaired immunity to encapsulated microbes
o Aplastic crisis: parvovirus B19
o Renal failure
o Cerebral: stroke, cognitive impairment
o Ocular and orbital complications
o Priapism
o Leg ulcers

ANSWER: Staph aureus infection is not associated with autosplenectomy

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12
Q

Which is false of CML? (March 2015)

a. Philadelphia chromosome
b. Transforms to AML
c. Bone marrow fibrosis
d. Thrombocytosis

A

CML:
Epidemiology: M>F, mean age 65 at Dx

Risk factors: Exposure to ionising radiation

Pathology: Philadelphia chromosome – t(9,22)

Classification:

  • Chronic phase: 85% at diagnosis (Mild symptoms, Progresses to accelerated phase if untreated)
  • Accelerated phase: 10-19% myeloblasts in BM, >20% basophils, Platelet count <100,000 or >1,000,000 unresponsive to therapy, splenomegaly or increased WBC
  • Blast crisis: Final phase, behaves like acute leukaemia, Rapid progression, short survival, >20% myeloblasts or lymphoblasts in BM or blood, Large clusters of blasts in the BM on biopsy, Development of choloroma

Bone lesions are permeative in nature

ANSWER: Bone marrow fibrosis is not typical of CML

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13
Q

Which is associated with the Philadelphia chromosome t(9;22)?

a. ALL
b. CLL

A

ANSWER: Philadelphia chromosome is most associated with CML, however can also be seen in ALL and AML.

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14
Q

In what situation is it most important to perform a core biopsy? (March 2016)

a. Lymph node with possible lymphoma
b. Lung cancer
c. Thyroid lesion
d. Lymph node with suspected secondary cancer when the primary cancer is known

A

ANSWER: A lymph node with possible lymphoma should have a core biopsy performed. The architecture of the neoplasm will determine the histological subtype. An FNA is inadequate for diagnosis.

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15
Q

Which is not associated with EBV? (March 2015)

a. Burkitt’s Lymphoma
b. NK/T cell lymphoma
c. Hodgkin Lymphoma
d. Plasmacytoma of the head and neck
e. Nasopharyngeal cancer

A
EBV related malignancies
o Haematological:
	Burkitt’s lymphoma
	Hodgkin’s disease
	NK/T cell lymphoma
	AIDS associated non-Hodgkin’s lymphoma
	Post-transplant lymphoma
o Nasopharyngeal Ca
o Gastric Ca
o Leiomyosarcoma

ANSWER: Plasmacytoma of the head and neck is not associated with EBV virus

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16
Q

What is implicated in the pathogenesis of plasmacytoma? (March 2014)

a. CMV

A

May be an association between EBV and multiple myeloma but this is not well established

ANSWER: CMV is not associated with the pathogenesis of multiple myeloma

17
Q

Which is false regarding multiple myeloma? (March 2017)

a. Predisposes to viral infections
b. Osteoporosis can occur in the absence of lytic lesions
c. Blood has high viscosity
d. Renal failure is a complication

A

ANSWER: Multiple myeloma predisposes the patient to bacterial infections, not viral infections

18
Q

Which of the following is not a type of Hodgkin’s lymphoma? (September 2013)

a. Anaplastic
b. Nodular sclerosing
c. Mixed cellularity
d. Lymphocyte rich
e. Lymphocyte depleted

A
Types of Hodgkin’s lymphoma:
o	Nodular sclerosing (most common)
o	Mixed cellularity
o	Lymphocyte rich
o	Lymphocyte depleted
o	Lymphocyte predominant

ANSWER: Anaplastic is not a type of Hodgkin’s lymphoma, but it is a type of non-Hodgkin’s lymphoma

19
Q

Which is true regarding Hodgkin’s lymphoma? (September 2013)

a. The Reed Sternberg cell is an abnormal B cell
b. Lymphocyte depleted is the most common subtype
c. Cannot be diagnosed by FNA

A

The Reed Sternberg cell
o Abnormal giant cells found in patients with HL
o Usually derived from abnormal B-lymphocytes (CD15 and CD30 immunomarkers)
o Large cells which are multinucleated or have a bilobed nucleus - Eosinophilic inclusion like nucleoli (“Owl’s eye” appearance)

Nodular sclerosing is the most common type of Hodgkin’s lymphoma

A diagnosis of lymphoma can be made with FNA, however this does not allow assessment of the architecture which enables typing
o Gold standard is an excision biopsy

ANSWER: The Reed Sternberg cell is an abnormal B cell; lymphoma should be diagnosed with a core biopsy

20
Q

Which is least correct regarding Waldenstrom macroglobulinaemia? (March 2014)

a. Bone lesions can be painful
b. Cold agglutinins are positive
c. Coombes test are positive

A
Waldenstroms macroglobulinaemia:
o	Positive direct Coombes
o	Positive cold agglutinins
o	Monoclonal IgM proliferation
o	Increased blood viscosity due to increased IgM titre

ANSWER: Bone lesions are not typically painful

21
Q

Epstein Barr virus is most associated with: (August 2016)

a. Natural killer NK/T cell leukaemia/lymphoma

A
EBV associated malignancies:
o	Burkitt’s lymphoma
o	B cell lymphoma
o	Hodgkin lymphoma (subset)
o	T cell/NK cell leukaemia/lymphoma
o	Nasopharyngeal cancer
o	Gastric cancer

ANSWER: NK/T cell leukaemia/lymphoma is associated with EBV

22
Q

Which lymphoma is least likely to be treated with curative intent? (March 2015)

a. Nodular sclerosing Hodgkin’s disease
b. Diffuse large B cell lymphoma
c. Mycosis fungoides
d. Follicular lymphoma
e. ALL

A
Mycosis fungoides (cutaneous T cell lymphoma) can be managed with chemotherapy, radiotherapy and immunotherapy but treatment goals are ultimately palliative
o	May be indolent in the skin for many years, before spreading to local nodes and then visceral organs including the lung and brain

Follicular lymphoma is a low grade neoplasm
o Can be chemo-resistant given the low grade, however the treatment goal is ultimately curative

ANSWER: Mycosis fungoides

23
Q

What is the most likely lesion to be negative on PET? (March 2014)

a. Nodular sclerosing Hodgkin disease
b. T cell lymphoma
c. Lymphocyte depleted lymphoma
d. Follicular B cell lymphoma

A

ANSWER: Out of these options, follicular B cell lymphoma has the least avidity but is still low grade. Other recalls list mycosis fungoides as the 5th option which is not PET avid and more correct. Technically – myocosis fungoides is a type of T cell lymphoma.

24
Q

Flow cytometry is most useful for which lesion? (March 2016)

a. Chloroma
b. Pancreatic adenocarcinoma

A

Granulocytic sarcoma (chloroma)
o Rare neoplasm of myeloid precursor cells
Focal accumulation of leukaemic cells

o Can occur in association with:
	AML
        CML
	Other myeloproliferative disorders:
•	Myelofibrosis with myeloid metaplasia
•	Hypereosinophilic syndrome
•	Polycythaemia vera

o Precedes the haematological disorder in 35% of cases

ANSWER: Chloroma – need to determine clonality of cells

25
Q

What is the most common complication of polycythaemia rubra vera? (August 2016)

a. Transformation to AML
b. Transformation to CML

A

Polycythaemia rubra vera:
- Myeloproliferative disorder which results in an excess of red blood cells in the bloodstream (Overproduction of erythrocytes, granulocytes and platelets)

Epidemiology: older populations with a slight male predilection

Pathology: 95% express the JAK2 mutation

Complications: Splenic infarction, Venous thrombosis (SMV, dural venous sinus, Budd Chiari syndrome), Cerebrovascular events, MI, Transformation to AML
• 2% in patients treated with phlebotomy
• 15% in patients treated with alkalizing agents

ANSWER: Of these options, transformation to AML is the most common. Splenic infarction would be the most common complication overall

26
Q

What is not associated with polycythaemia rubra vera? (March 2014)

a. Cyanosis
b. Thrombocytosis

A

ANSWER: Cyanosis is not associated with PRV, the patients appear plethoric

27
Q

Which of the following is the least likely association? (March 2014)

a. Autoimmune haemolytic anaemia is associated with thrombotic thrombocytopaenic purpura
b. HUS can occur in pregnancy
c. Gallstones are associated with polycythaemia vera

A
Thrombotic microangiopathies:
o	TTP and HUS:
Characterised by:
•	Thrombocytopaenia
•	Microangiopathic haemolytic anaemia
•	Fever
•	Transient neurological deficits (TTP)
•	Renal failure (HUS)  
Clinically similar to DIC, however caused by excessive platelet activation rather than activation of the clotting cascade

Pathology:
• Widespread hyaline microthrombi in arterioles and capillaries
• Dense aggregates of platelets and fibrin

Treatment:
• Supportive for typical HUS
• Plasmaphoresis and steroids

o Thrombotic thrombocytopaenic purpura
- Caused by inherited or acquired deficits in ADAMTS13

o Haemolytic uraemic syndrome
- Typical HUS follows gastrointestinal infection with E. Coli
• Toxins injure endothelial cells and results in dysregulated platelet activation
• Occurs in children and elderly
• Associated with ‘food poisoning’ outbreaks
- Atypical HUS is caused by defects in proteins which prevent excessive activation of the alternative complement pathway
• Inherited defects or acquired auto-antibodies
• Associated with connective tissue diseases (e.g. SLE) and malignancies
• Pregnancy associated haemolytic uraemic syndrome

ANSWER: Polycythaemia vera does not appear to be associated with gallstones. It is more commonly associated with manifestations of hyperuricaemia from increased turnover of reticulocyte nuclei and purine degradation, leading to an accumulation of uric acid in the blood. Patients may present with gout.

28
Q

What is the most common risk factor for thymoma? (August 2016)

a. EBV
b. Radiation for Hodgkin’s lymphoma
c. Chemotherapy

A

Thymic epithelial tumours
Epidemiology: 5-6th decades, no gender predominance

The only confirmed risk factors for thymoma are age and ethnicity (Asian, Polynesian and African Americans)

o	Associated conditions (over 30 known):
	Myasthenia gravis (most common) (30-50%%)
	Pure red cell aplasia (50%)
	Hypogammaglobulinaemia (10%)
	SLE
	Rheumatoid arthritis
	Non-thymic cancers

WHO classification for thymic cancers:
o Type a: tumours that are cytologically benign and non-invasive
o Type b: tumours that are cytologically benign but invasive or metastatic
o Type c: tumours that are cytologically malignant (thymic carcinoma)

ANSWER: None of these risk factors are common for thymoma – some research has suggested links with radiation and EBV but this is unverified. The known risk factors are increasing age and ethnicity.

29
Q

Regarding thymoma in adults, which is true? (March 2017)

a. The most common thymic abnormality is hyperplasia
b. Pleural spread is usually contiguous

A
True thymic hyperplasia:
o	Rebound hyperplasia secondary to chemotherapy or steroid use
o	Radiation therapy
o	Burns
o	Other severe systemic stresses
Thymic lymphoid hyperplasia:
o	Myasthenia gravis
o	SLE
o	Rheumatoid arthritis
o	Scleroderma
o	Graves disease

ANSWER: Pleural spread is usually contiguous.

30
Q

Iron overload is LEAST accepted to occur in which of the following settings?

  1. Increased absorption of dietary iron due to an inborn error of metabolism
  2. Haemolytic anaemia
  3. Repeated blood transfusion over many years
  4. Chronic renal failure with urine alkalinisation
  5. Chronic Ingestion of food / drink exposed to iron pots (“bantu siderosis”)
A
  1. *Chronic renal failure with urine alkalinisation
31
Q

A patient has irregularity / possible clot of the cardiac valves. Which of the following is LEAST likely to be a cause?

  1. Hypercoagulable state
  2. Post mortem finding
  3. Systemic Lupus erythematous
  4. Advanced cachexia
  5. Hepatic encephalopathy
A
  1. *Hepatic encephalopathy
32
Q

Which of the following is LEAST likely to associated with haemorrhagic (red) infarct?

  1. Testicular torsion
  2. Pulmonary infarct
  3. Small bowel infarcts secondary to occluded SMV
  4. Splenic infarct secondary to aortic atheroma
  5. Cerebral infarct after clot extraction
A
  1. *Splenic infarct secondary to aortic atheroma
33
Q

Concerning HIV infection, which of the following statement is most correct?

  1. HIV RNA in blood reflect infection with the virus but levels are independent of viral load/disease activity
  2. A Low CD4+ count reflects T cell suppression: there is no increased risk to conventional capsulated bacterial infection
  3. A CXR request stating “Fever. Acute HIV / “Acute Retroviral Syndrome” indicates a high risk of active Pneumocystis jiroveci pneumonia
  4. 25 to 40% of untreated patients develop a malignancy
  5. Chronic diarrhea is most commonly due to indolent CMV infection / “chronic typhlitis”
A
  1. *25 to 40% of untreated patients develop a malignancy
34
Q

A haematology patient has a request stating “CT HEAD: Mucormycosis”. Which of the following statements is LEAST correct?

  1. Mucormycosis is an aggressive fungal infection commonly affecting the paranasal sinuses – if there are no other scans, the sinuses +/- need for IV Contrast should be assessed.
  2. Mucormycosis infection most commonly causes a bland chronic basal meningitis (akin to cryptococcus)
  3. Patients at higher risk of infection include immunosuppressed, and patients with iron chelation therapy and diabetic patients
  4. The most common sites of infection are the nasal sinuses (+/- adjacent structures), lungs and GIT.
  5. Mucormycosis can invade arterial walls leading to thrombosis and local infarction
A
  1. *Mucormycosis infection most commonly causes a bland chronic basal meningitis (akin to cryptococcus)
35
Q

Concerning Disseminated Intravascular Coagulation (DIC) which of the following is the LEAST correct?

  1. It may be seen in advanced malignancy without infection
  2. It may be in obstetric setting without infection
  3. It is characterised by excessive bleeding
  4. It characteristically spares the brain and lungs
  5. The Platelet Count is usually reduced
A
  1. *It characteristically spares the brain and lungs
36
Q

Concerning infarcts which of the following are most correct?

  1. They may occur in organs which have patent arteries and veins
  2. They are due to intrinsic/ luminal causes rather than extrinsic compression
  3. Impaired venous drainage causes congestion but does not cause infarction in the absence of pre-existing atheroma
  4. Over 65% of all deaths in the USA are due to CVA or MI
  5. Red infarcts are characteristic of the kidney
A
  1. *They may occur in organs which have patent arteries and veins