Haematology Flashcards
(36 cards)
Which is a cause of platelet dysfunction? (August 2014)
a. Splenomegaly
b. Uraemia
ANSWER: Uraemia
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
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
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
ANSWER: Determining the clonality of lymphomas
Man with HIV presenting with multiple small lesions in the liver and spleen. Most likely?
a. Candida
b. Lymphoma metastases
Hepatosplenic candidiasis
o Multiple small hypodense lesions in the liver and spleen
o Immunosuppressed patients (e.g. HIV, chemotherapy)
ANSWER: Candida
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
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
Splenectomy increases susceptibility to which bacteria? (August 2014)
a. H. influenzae
b. Tuberculosis
c. Mycosis fungoides
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
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
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
Which does not cause splenomegaly? (March 2017)
a. Biliary ascariasis
b. Schistosomiasis
c. Malaria
ANSWER: Biliary ascariasis
Which is least likely to result in splenomegaly? (March 2017)
a. Hepatitis B
b. Budd Chiari
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
Splenic infarcts are most commonly from: (March 2015)
a. Sickle cell
b. Spherocytosis
c. Thalassaemia
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
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
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
Which is false of CML? (March 2015)
a. Philadelphia chromosome
b. Transforms to AML
c. Bone marrow fibrosis
d. Thrombocytosis
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
Which is associated with the Philadelphia chromosome t(9;22)?
a. ALL
b. CLL
ANSWER: Philadelphia chromosome is most associated with CML, however can also be seen in ALL and AML.
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
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.
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
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
What is implicated in the pathogenesis of plasmacytoma? (March 2014)
a. CMV
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
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
ANSWER: Multiple myeloma predisposes the patient to bacterial infections, not viral infections
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
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
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
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
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
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
Epstein Barr virus is most associated with: (August 2016)
a. Natural killer NK/T cell leukaemia/lymphoma
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
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
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
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
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.
Flow cytometry is most useful for which lesion? (March 2016)
a. Chloroma
b. Pancreatic adenocarcinoma
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
