Flashcards in Haematology Deck (36)
Match the following observation on peripheral blood film to it's key underlying condition.
(a) Heinz Bodies
(b) Howell-Jolly bodies
(c) Pelger Huet cells
(1) Myelogenous leukaemia and myelodysplatic syndromes
(2) Increased in haemolytic anaemias and decreased in aplastic anaemias.
(3) Glucose-6-phosphate dehydrogenase deficiency
(4) Microangiopathic anaemia, e.g. DIC, haemolytic-uraemic syndrome and thrombotic thrombocytopenic purpura
(5) Post-splenectomy or hyposplenism, megaloblastic anaemia.
The correct matches are as following:
(a) Heinz bodies are inclusions within red blood cells of denatured haemoglobin and are seen in (3) Glucose-6-phosphate dehydrogenase deficiency as well as chronic liver disease.
(b) Howell-Jolly bodies are basophilic (purple spot) nuclear remnants in red blood cells which are seen in (5) Post-splenectomy or hyposplenism, megaloblastic anaemia, classically seen in sickle cell disease.
(c) Pelger Huet cells are hyposegmented neutrophils which are seen in (1) Myelogenous leukaemia and myelodysplatic syndromes. These conditions are an acquired cause of Pelger Huet cells, there is also a congential cause which is due to a Lamin B receptor mutation.
(d) Reticulocytes are immature red blood cells which are (2) Increased in haemolytic anaemias and decreased in aplastic anaemias. They're also reduced in patients receiving chemotherapy.
(e) Schistocytes are fragmented parts of red blood cells which are typically irregularly shaped, jagged and asymmetrical, and they are seen in (4) Microangiopathic anaemia, e.g. DIC, haemolytic-uraemic syndrome and thrombotic thrombocytopenic purpura.
Which of the following conditions can cause macrocytic anaemia?
(a) Iron deficiency anaemia
(b) Anaemia of chronic disease
The correct answer is (c) Hypothyroidism. Hypothyroidism is a cause of either normocytic or macrocytic anaemia.
Iron deficiency anaemia (a) causes a microcytic anaemia.
Anaemia of chronic disease (b) can cause a microcytic or normocytic anaemia.
Haemolysis(d) and pregnancy (d) are both causes of normocytic anaemia.
Your reg asks you to interpret a plasma iron study from a patient with sideroblastic anaemia. What pattern would you expect to see in the iron level, total iron binding capacity(TIBC) and ferritin?
(a) Iron low, TIBC high, and ferritin low.
(b) Iron low, TIBC low, and ferritin high.
(c) Iron high, TIBC low, and ferritin high
(d) Iron high, TIBC normal, and ferritin high
(e) Iron high, TIBC high, and ferritin normal
The correct answer is (d) Iron high, TIBC normal, and ferritin high.
(a) Iron low, TIBC high, and ferritin low is the pattern you would see in iron deficiency.
(b) Iron low, TIBC low, and ferritin high is the pattern you would see in anaemia of chronic disease.
(c) Iron high, TIBC low, and ferritin high is the pattern you would see in chronic haemolysis.
(e) Iron high, TIBC high, and ferritin normal is the pattern you would see in pregnancy.
A 34 year old woman is brought in by her concerned husband. He reports she's become increasingly irritable and low in mood over the past 5 months, and recently she's been responding to unseen stimuli. On examination she has angular cheilosis, and noticeable loss of vibration sense and proprioception. Her husband says she doesn't drink any alcohol, and that they both eat a healthy, balanced, vegan diet. Her peripheral blood film shows hypersegmented polymorphs, leucopenia, anaemia and thrombocytopenia.
What is the cause of this woman's symptoms?
(a) Folate deficiency
(b) Cobalamin deficiency
(c) Thiamin deficiency
(d) Niacin deficiency
(e) Ascorbate deficiency
The correct answer is (b) Cobalamin deficiency. Cobalamin is vitamin B12 and a vitamin B12 deficiency causes all the symptoms on show in this woman, as well as glossitis, dementia and paraethesiae. The history points towards a megaloblastic anaemia, and veganism indicates a B12 deficiency as it's found in meat and dairy products.
Folate deficiency (a) causes a megaloblastic, macrocytic anaemia. Causes of a folate deficiency are poor diet (folate is found in green vegetables, nuts, yeast and liver), increased demand (pregnancy of increased cell turnover such as in haemolysis or malignancy), malabsorption (coeliac disease, tropical sprue) and drugs (alcohol, anti-epileptics such as phenytoin, methotrexate and trimethoprim).
Thiamin (c) is vitamin B1, and a deficiency causes Beri-Beri, neuropathy and wernicke's syndrome.
A deficiency in Niaccin (d) causes Pellagra.
Ascorbate (e) is vitamin C, a deficiency of which causes scurvy. An excess can cause renal stones.
Which of the following statements about hereditary spherocytosis is incorrect?
(a) Autosomal dominant is the main form of inheritance.
(b) Due to either a spectrin or ankyrin deficiency
(c) Causes an increased susceptibility to parvovirus B19.
(d) Diagnosis is made by presence of spherocytes decreased osmotic fragility and a negative DAT test.
(e) Treatment is splenectomy and folic acid.
The correct answer is (d) Diagnosis is made by presence of spherocytes decreased osmotic fragility and a negative DAT test. Instead there is increased osmotic fragility which is when the cells lyse in hypotonic solutions.
A 3 month boy is brought into A&E. He is severely anaemic with clear failure to thrive, on examination he has hepatosplenomegaly and signs of heart failure.
What thalassaemia variant gives rise to this presentation?
(a) β - thalassaemia minor
(b) β - thalassaemia major
(c) α - thalassaemia trait
(d) Haemoglobin H disease
(e) α - thalassaemia major
The correct answer is (b) β - thalassaemia major.
(a) β - thalassaemia minor is a heterozygous, asymptomatic carrier, sometimes has mild anaemia.
(c) α - thalassaemia trait has 1/2 α globin gene deletions and is mostly asymptomatic, with an occasional mild anaemia.
(d) Haemoglobin H disease occurs when three α globin genes are deleted, and presents in childhood or early adult life with moderate anaemia and splenomegaly.
(e) α - thalassaemia major occures when all four α globin genes are deleted, and is strongly incompatible with life, causing hydrops foetalis. If known about very early on there is a possibility of intrauterine transfusions and either a bone marrow transplant after delivery or chronic transfusions.
Which of the following is not a cause of warm acquired haemolytic anaemia?
(e) EBV infection
The correct answer is (e) EBV infection. This is a cause of cold agglutinin disease instead.
Lymphoma (a) can cause warm acquired haemolytic anaemia and cold agglutinin disease.
SLE (b), CLL (c) and Methyldopa (d) are all causes of warm acquired haemolytic anaemia.
A 32 year old woman who is 18 weeks pregnant presents to A&E with jaundice and abdominal pain. On examination there are ascites and hepatomegaly. She reports that her urine has been dark red the past few mornings. Her haemoglobin is 8.6, and her lactate and bilirubin are both raised.
What is causing this woman's condition?
(b) Venous thrombosis
(c) Thrombotic thrombocytopenic purpura
(d) Normal pregnancy
(e) Paroxysmal nocturnal haemoglobinuria
The correct answer is (e) Paroxysmal nocturnal haemoglobinuria. The constellation of symptoms point to PNH complicated with budd-chiari syndrome. 25% of women with PNH are diagnosed during pregnancy, as the naturally hyper-coaguable state exacerbates the underlying condition.
Pre-eclampsia (a) can cause microangiopathic haemolytic anaemia which would account for the haemolytic picture of her bloods, but it wouldn't cause budd-chiari syndrome, nor haemoglobinuria.
Venous thrombosis (b) is causing her acute presentation, of budd-chiari syndrome, however it does not account for the haemolytic picture of her bloods, nor haemoglobinuria. Only PNH accounts for the whole picture.
Which factor is involved in the extrinsic coagulation pathway?
The correct answer is (a) Seven.
The others are all factors involved in the intrinsic pathway, along with twelve which is the first factor in the pathway.
Which of the following statements about acute auto-immune thrombocytopenic purpura is correct?
(a) Affects females:males in a 3:1 ratio.
(b) Preceding infection is rare
(c) Peak incidence is in adulthood
(d) Usual duration is 2-6 weeks
(e) Spontaneous remission is uncommon.
The correct answer is (d) Usual duration is 2-6 weeks.
All of the other statements describe chronic auto-immune thrombocytopenic purpura.
Acute ITP affects children, between 2-6 years old is the peak incidence. Females:males is 1:1, and preceding infection is common. Onset of symptoms is abrupt, platelet count at presentation is <20000, duration is 2-6 weeks and it is usually self-limiting.
A set of bloods show a highly raised INR, APTT and thrombin time, as well as a raised D-dimer.
What bleeding disorder would give rise to these results?
(a) Vitamin K deficiency
(d) von Willebrand's disease
(e) Heparin use
The correct answer is (b) DIC. In addition to these findings you would also see reduced platelets and increased bleeding time.
In vitamin K deficiency (a) there would be a high INR, a raised APTT and deficiencies in factors II, VII, IX, X and protein C/S.
In Haemophilia (c) there would be a raised APTT and there would be reduced factor VIII in A and reduced factor IX in B.
von Willebrand's disease (d) would show a raised APTT and an increased bleeding time, as well as decreased factor VIII and decreased vWF Ag.
Heparin use (e) would cause a slightly increased INR, a raised APTT and raised thrombin time.
Sort the following prognostic factors in chronic lymphocytic leukaemia into good factors and bad factors.
(a) Raised LDH
(b) Hypermutated Ig gene
(c) Low ZAP-70 expressions
(d) CD38 positive
(e) 11q23 deletion
(f) 13q14 deletion
The good prognostic factors are (b) hypermutated Ig gene, (c) Low ZAP-70 expressions and (f) 13q14 deletion.
The bad prognostic factors are (a) Raised LDH, (d) CD38 positive and (e) 11q23 deletion
Which of the following is not part of the clinical presentation of Hodgkin's lymphoma?
(a) Symmetrical, painful lymphadenopathy
(b) Weight loss
(d) Low grade fever
(e) Pain in affected nodes after alcohol
The correct answer is (a) symmetrical, painful lymphadenopathy. The lymphadenopathy in Hodgkin's lymphoma is asymmetrical and painless.
A CT of a patient with suspected Hodgkin's lymphoma showed mediastinal and inguinal lymph node involvement. The history stated the patient had experienced weight loss, low grade fever, night sweats, pruritis and fatigue.
What stage is this patient's Hodgkin's lymphoma?
(a) Stage 2a
(b) Stage 2b
(c) Stage 3a
(d) Stage 3b
(e) Stage 4a
The correct answer is (b) stage 2b.
There are two aspects to staging Hodgkin's lymphoma, numbers and letters.
The numbers correspond to the spread of disease.
Stage 1 - One lymph node region (including spleen)
Stage 2 - Two or more lymph node regions on the same side of the diaphragm
Stage 3 - Two or more lymph node regions on on opposite sides of the diaphragm
Stage 4 - extranodal sites (liver, bone marrow)
In this patient there were two lymph node regions involved on the same side of the diaphragm, so it's stage 2.
Whether it is a or b is simply whether there are constitutional symptoms present or not. If not, it's a, if they are present it's b.
They are present in this patient so the final staging would be 2b.
Which of the following is not part of combination chemotherapy for Hodgkin's lymphoma?
The correct answer is (a) Rituximab. Rituximab is the mainstay of treatment for the B cell lymphomas.
Combination chemotherapy is used in most cases and consists of ABDV: Adriamycin, Bleomycin, Dacarbazine and Vinblastine. (Current number 1 ranked batsman in the world right now is South African A.B. de Villiers. Idk it helped me)
Which of the following statements about T-cell lymphomas is incorrect?
(a) Anaplastic large cell lymphoma affects children and young adults, is aggressive, has large "epithelioid" lymphocytes, associated with t(2;5) and expression of the protein anaplastic lymphoma kinase (Alk-1)
(b) Peripheral T-cell lymphoma affects the middle-aged and elderly, is aggressive and is characterised by large T-cells.
(c) Adult T-cell leukaemia/lymphoma affects the caribbean and japanese population especially, is caused by HTLV-1 infection, aggressive.
(d) Enteropathy-associated T cell lymphoma (EATL) is a marginal zone non-hodgkin's lymphoma which affects the middle-aged and is caused by chronic antigen stimulation such as H. pylori and Sjogren's syndrome.
(e) Cutaneous T cell lymphoma is associated with mycosis fungoides and can be treated by Alemtuzumab which is anti CD-52.
The correct answer is (d) Enteropathy-associated T-cell lymphoma (EATL) is a marginal zone non-hodgkin's lymphoma which affects the middle-aged and is caused by chronic antigen stimulation such as H. pylori and Sjogren's syndrome.
This answer instead describes mucosal associated lymphoid tissue (MALT) which is a B-cell lymphoma.
EATL is associated with longstanding coeliac disease.
Alemtuzumab, mentioned in (e) is actually an appropriate treatment for all the T-cell lymphomas.
The standard first-line treatment for non-Hodgkin's lymphoma is the R-CHOP regimen. Which of the following parts of that regimen are incorrect?
The correct answer is (b) Cyclosporin. Instead Cyclophosphamide is the C component
Cyclophosphamide is an alkylating agent which damages DNA by binding to it and causing the formation of cross-links.
Hydroxydaunorubicin (also called doxorubicin or adriamycin) is an intercalating agent which damages DNA by inserting itself between DNA bases.
Oncovin (vincristine), which prevents cells from duplicating by binding to the protein tubulin.
Prednisolone is the one component of therapy given for 5 days, the rest are just 1 day of therapy.
Affects middle aged, M>F. Aggressive and disseminated at presentation. Median survival 3-5 years. Associated with t(11;14) translocation and cyclin D1 deregulation. Histology shows angular nuclei.
What condition does this describe?
(a) Sporadic Burkitt's lymphoma
(b) Endemic Burkitt's lymphoma
(c) Diffuse Large B-cell
(d) Mantle Cell Lymphoma
(e) Follicular Lymphoma
The correct answer is (d) Mantle cell lymphoma.
Burkitt's lymphomas are all very aggressive, fast-growing, t(8;14) translocation, with c-myc oncogene overexpression, and is rapidly responsive to treatment. Histology is classically the starry sky appearance.
Specifically, sporadic burkitt's (a) is found outside Africa, EBV-associated but jaw less commonly involved, whereas endemic burkitt's (b) is the most common malignancy in equatorial Africa, also EBV associated but with characteristic jaw involvement and abdominal masses.
Diffuse large B-cell (DLBC) (c) affects the middle-aged and elderly, aggressive, Richter's transformation accounts for some of the cases - Richter's is when CLL transforms into DLBC. Other lymphomas occur secondary to DLBC. On histology there's sheets of large lymphoid cells.
Follicular lymphoma (e) is indolent, mostly incurable, with a median survival of 12-15 years, associated with t(14:18) translocation. On histology there is a follicular pattern or nodular appearance.
Which of the following investigation results for multiple myeloma is incorrect?
(a) Broad band on serum electrophoresis
(b) Rouleaux on blood film
(c) Bence-Jones protein in urine
(d) Very high ESR
(e) >10% plasma cells in bone marrow
The correct answer is (a) broad band on serum electrophoresis. A broad band would indicate polyclonal, in multiple myeloma you would see a dense narrow band.
Which of the following is not a classical clinical feature of multiple myeloma?
(b) Renal failure
The correct answer is (a) hypocalcaemia. Instead multiple myeloma causes a hypercalcaemic state.
Which of the following is not a classical clinical feature of myelodysplastic syndromes?
(a) Bone marrow failure and cytopenias - causing infection, bleeding and fatigue
(b) Hypercellular bone marrow
(c) Red blood cell defects like ring sideroblasts which are abnormal nucleated blast surround by an iron granule ring.
(d) White blood cell defects like Pelger-Huet anomaly, which are hypersegmented neutrophils.
(e) Platelet defects, with micromegakaryocytes and hypolobulated nuclei
The correct answer is (d) White blood cell defects like Pelger-Huet anomaly, which are hypersegmented neutrophils.
There are two factors that are in correct in this statement. Pelger-Huet anomaly occurs in a genetic disorder of the Lamin B receptor, and it causes a hyposegmented neutrophil not hypersegmented. Because of this, even though the cells are the same, they are referred to as pseudo-pelger-huet cells in myelodysplastic syndromes.
Which of the following set of blood features and bone marrow features describes myelodysplastic syndrome with 5q deletion?
(a) Blood features: Anaemia, no blasts. Bone marrow features: Erythroid dysplasia with <5% blasts
(b) Blood features: Cytopenia in ≥ 2 cell lines. Bone marrow features: Dysplasia in >10% cells in ≥2 cell lines.
(c) Blood features: Cytopenias, <5% blasts, no Auer rods. Bone marrow features: Dysplasias, 5-9% blasts.
(d) Blood features: Anaemia, normal or increased platelets. Bone marrow features: megakaryocytes with hypolobulated nuclei and <5% blasts.
(e) Blood features: complex - cytopenias, no blasts, no Auer rods. Bone marrow features: complex - myeloid or megakaryotic dysplasia, <5% blasts.
The correct answer is (d) Blood features: Anaemia, normal or increased platelets. Bone marrow features: megakaryocytes with hypolobulated nuclei and <5% blasts.
(a) describes Refractory anaemia. The addition of >15% ringed sideroblasts in the bone marrow would give you the picture of refractory anaemia with ringed sideroblasts.
(b) describes refractory cytopenia with multilineage dysplasia (RCMD). An addition of >15% ringed sideroblasts in the bone marrow would give you RCMD and ringed sideroblasts.
(c) Describes refractory anaemia with excess blasts - 1 (RAEB I). The difference between type 1 and type 2 is a higher level of blasts in either the blood or bone marrow (5-19% in blood 10-19% in bone marrow) or the presence of Auer rods.
(e) Describes myelodysplasia syndrome - unclassified.
Which of the following agents has a delayed anticoagulant effect?
(a) Vitamin K
(b) Unfractionated Heparin
The correct answer is (c) Warfarin.
Female aged 39 previous treated for breast cancer 4 years ago presents with recent onset jaundice, hepatomegaly and backache. Blood results show Hb of 87, bilirubin of 5, DAT negative and the peripheral blood film shows an leuko-erythroblastic picture.
What is the likely cause of this anaemia?
(a) Iron deficiency anaemia
(b) Anaemia of chronic disease
(c) Bone marrow metastases
(d) Acute myeloid leukaemia
(e) Auto-immune haemolytic anaemia.
The correct answer is (c) Bone marrow metastases.
In which of the following infections would you expect to see a raised basophil count?
(c) Pox viruses
(e) All of the above
The correct answer is (c) Pox viruses.
Which of the following statements is correct?
(a) Telomeric shortening is a feature of both idiopathic aplastic anaemia and dyskeratosis congenita
(b) Development of malignancy is an uncommon complication of Fanconi's Anaemia
(c) A single genetic defect has been identified as the underlying cause for Fanconi's Anaemia
(d) Fanconi's Anaemia is usually inherited in an autosomal dominant fashion.
(e) Telomeric function is considered to be unimportant in the pathophysiology of Dyskeratosis Congenita.
The correct answer is (a) Telomeric shortening is a feature of both idiopathic aplastic anaemia and dyskeratosis congenita.
Untreated HIV infection is associated with which of the following types of lymphoma?
(a) Gastric MALT lymphoma
(b) High grade B cell lymphoma
(c) Parotid gland MALT lymphoma
(d) T cell lymphoma of skin
(e) Acute T cell leukaemia lymphoma
The correct answer is (b) High grade B cell lymphoma. This is due to EBV infection in an immunosuppressed individual.
53 year old male with weight loss and abdominal pain. On examination there is hepatomegaly and massive splenomegaly. Hb 98, WBC 20 and platelets 60. The peripheral blood film shows an erythroblastic picture. BCR-ABL transcripts not detected. JAK2 V617F 20%. BM aspirate dry tap.
What is the cause of these findings?
(a) CML chronic phase
(b) CML blast crisis
(c) Bone marrow metastases from prostate cancer
(d) Primary myelofibrosis
(e) Hodgkin's lymphoma
The correct answer is (d) Primary myelofibrosis. Leukoerythroblastic blood film suggest fibrosis in the bone marrow, not just a CML picture. This added to the JAK2 mutation and the dry tap on BM aspiration completes a picture of primary myelofibrosis.
Bone marrow metastases (c) is wrong, massive splenomegaly is rare, there wouldn't be an acquired JAK2 mutation.
CML chronic phase (a) would have a very high Hb and platelet count. CML blast crisis (b) fits the bloods but BCR-ABL negative shows it isn't CML in either case.
22 year old male with cyanotic congenital heart disease. Hb is 210 and haematocrit 60%. Frusemide 160mg daily. Serum erythropoietin levels and JAK2 mutation analysis is ordered.
What picture do you expect to see in this patient?
(a) JAK2 V617F negative, low serum Epo level
(b) JAK2 V617F positive, relative polycythaemia
(c) JAK2 V617F negative, raise serum Epo level
(d) JAK2 V617F positive, low serum Epo level
The correct answer is (c) JAK2 V617F negative, raise serum Epo level.
This is a polycythaemia, in a 22year old with cyanotic congenital heart disease and results that fit with polycythaemia. This points towards a true polycythaemia, the main drive being the cyanotic heart disease. Next part of the question is asking whether it is malignant polycythamia vera and by extension JAK2 V617F positive or is it secondary polycythaemia because of cyanosis, hypoxia driven erythropoiesis. In this case it is the latter.