"Best Of Five Questions For MRCP UK And Ireland Examinations" Dr. Osama Amin MBChB, MRCPUK part I,MRCPI part I ,MRCPI part II written Senior House Officer and 4th year student of the…..Board of Neurology. Dedication to The Republic Of Iraq and Iraqi Doctors all over the world
Q1 Differential diagnosis of Iron deficiency anemia ……all are true except: 1-aluminuim toxicity 2-beta thalassemia syndromes 3-alpha thalassemia syndromes 4- Lead poisoning 5-aplastic anemia
Answer: 5 1-important in dialysis patients and causes resistance to Erythropoietin therapy 2-especially the minor trait 3-especially the minor trait, a clue that the patient is an Asian one with normal hemoglobin electrophoresis and normal ferritin 4-and sideroblastic anemias :clue is a dimorphic blood picture Remember: Microcytosis and hypochromia have a relatively narrow differential diagnosis so you have to know it because it is a common clinical problem. Aplastic anemia causes either a normocytic or macrocytic anemia
Q2 Causes of macrocytosis without a megaloblastic bone marrow, all are true except 1-myelodysplastic syndromes 2-aplastic anemia 3-multiple myeloma 4-chronic myeloid leukemia 5-fish tapeworm infestation
Answer 5 Remember: macrocytosis is very common in clinical practice but megaloblastic anemia is uncommon and the term macrocytosis indicates an increase in the MCV but megaloblastic changes are pathological changes seen in the cells mainly in the nucleus. Fish tapeworm infestation causes B12 deficiency and hence megaloblastic changes Note the first 4 cause characteristic changes in the bone marrow
Q3 Causes of macrocytosis with normal bone marrow, all are true except : 1-liver disease 2-neonatal 3-pregnancy 4-post –hemorrhage 5-myeolmatosis
Answer5 Item 5 causes characteristic changes in the bone marrow not a normoblastic one
Q4 Causes of dimorphic blood picture …all are true except : 1-a patient with long standing iron efficiency anemia who had received blood transfusion because of symptomatic coronary artery disease 2-long standing iron deficiency anemia patient who had received iron therapy before 1 week 3-celiac disease patient incompliant with glutin free diet 4-bone marrow study of a patient showing characteristic rings in the maturing erythroid series 5-a young pregnant lady on long term treatment with phenytoin
Answer 5 Dimorphic blood picture is very common problem seen in clinical medicine. It has 4 causes in general that you HAVE TO KNOW: 1-blood transfusion to a patient with pre-existent macro- or microcytosis 2-in the way of recovery of hematinic deficiency (i.e. during its replacement) 3-combined deficiency states, simultaneously or in succession .e.g. celiac disease 4-primary sideroblastic anemia where there is 2 CLONES one is the normal and the other is the abnormal one producing the macrocytes
In item 5, phenytoin causes macrocytosis exacerbated by pregnancy.
Q5 Sickle cell anemia, all are true, except: 1-despite the degree of anemia in the chronic stable state it is usually rarely symptomatic 2-in contrast to beta thalassemia major, blood transfusion is rarely indicated except probably in crisis or in certain selected patients not in crisis 3-the target of blood transfusion is to raise the hemoglobine A to 85-90% of the total hemoglobins and the PCV should be between 30-36 % 4-pulmonary infarction-chest syndrome is the commonest cause of death in adults 5-stroke in general is seen in 70 % of adults
Answer 5 1-true .usually tolerated because of the rightward shift in oxyhemoglobin dissociation curve 2-true regular blood transfusion are uncommonly indicated except in those with recurrent life threatening crisis like recurrent stroke 3-true, the objective is to reduce the HbS ,NOT TO RAISE THE PCV which if high may induce stagnation and further sickeling!!!! So be careful 4-true,unfortunately. 5-false up to 10% of children
Q6 Beta thalassemias, all are true except 1-Hb A2 may be normal or even low in beta thalassemia minor 2-regular transfusion is the mainstay in the treatment of beta thalassemia major 3-beta thalassemia minor usually comes into light after a failure of oral iron therapy for a mild iron deficiency anemia 4-hear failure in beta thalassemia major is only due to iron overload causing cardiomyopathy 5-target cells are seen in peripheral blood film
Answer 4 1-TRUE. be careful as there may be a co-existent iron deficiency anemia causing FALSLY low or normal HbA2 2-together with iron chelation therapy and daily folic acids 3-the usually story and then confirmed by hemogl;obin electrophoresis 4-false may be due to severe longstanding anemia causing high out put cardiac failure 5-and profound dyserythropoiesis
Q7 Causes of prominent intravascular hemolysis, all are true except 1-falciparam malaria 2-clostridia septicemia 3-immediate major ABO incompatibility reaction 4-paroxysmal nocturnal hemoglobinuria 5-hemolytic disease of the new born
Answer 5 Hemolytic disease of the new born causes mainly extra vascular hemolysis
Q8 Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) 1- neurological manifestations are more prominent in TTP 2- renal impairment is more prominent in HUS 3- skin rash is mainly seen in TTP 4- prolonged PT and aPTT occurs in HUS rather than TTP 5- vincristin may be used in the treatment
Answer 4 1-any neurological syndromes + fever + skin rash =exclude TTP 2-true for unknown reason 3-prupuric rash or frank ecchymosis 4-false impaired coagulation systems (PT and aPPT) indicate DIC, these are normal in pure HUS or TTP 5-to prematurely release platelets from the bone marrow
Q9 Causes of eosinophilia, all are true, except: 1-psoriasis 2-trichinosis 3-treatment with steroids 4-Hodgkin’s disease 5-sarcoidosis
Answer 3 1-and eczema and other skin diseases 2-filiariasis, ascariasis 3-Treatment with steroids causes neurtrophilia and eosinopenia 4-and CML 5-PAN and esosinophilic granuloma
Q10 Causes of monocytosis, all are true except 1-bone marrow recovery from chemotherapy 2-sarcoidosis 3-myelodysplastic syndrome 4-Kala-azar 5-hairy cell leukemia
Q11 Causes of neurtropenia, all are true except 1-racial 2-associated viral infections 3-hyperspelenism 4-treatment with carbimazole 5-leptospirosis
Answer 5 1-as in Arabs and blacks 2-like hepatitis A 3-ususally associated with low platelets 4-and other drugs like penicillamine 5-false causes neutrophila which is useful clue to differentiate it from viral hepatitis.
Q12 Poor prognostic factors in acute lymphonlastic leukemia, all are true except 1-male sex 2-age less than 1 year and more than 10 years 3-CNS involvement at the time of diagnosis 4-common ALL type 5-very high leukocyte count at the time of diagnosis
Answer 4 The common ALL type has a good prognosis .It is T cell type that presents with a mediastinal mass and a very high leukocyte count and has a worse prognosis Also t (9, 22) cytogenetic abnormality has a very poor prognosis (unlike CML where its presence confers a good prognosis)
Q13 Which one of the followings cytogenetic abnormalities is seen in acute myeoloid leukemia 1-M6 subtype–inv 16 2-M3 subtype–t(15,17) 3-M5 subtype- t( 8,21) 4-M1 subtype- t(1,15) 5-M2 subtype-t ( 2,14)
Answer 2 Knowing the cytogentics of AML is very important, not only for the prognosis but also to decide the future treatment like bone marrow transplantation in those with poor cytogenetics M2-t(8,21) M3-t(15,17) M4-inv16 Also in ALL: t(1,19) in pre-B ALL, t(8,14) ALL L3 Burkitt’s subtype
Q14 AML M3 subtype, all are true except 1-t(15,17) confers a good prognosis 2-the genetic defect lies in RARA gene 3-treatment with ATRA enhances further differentiation of the promyeloblasts from its differentiation block site 4-almost always of a hypogranular type 5-evidence of DIC is present up to 80% at the time of diagnosis
Answer 4 1-true 2-Retenoic Acid Receptor Alpha 3-true, hence rapid neurtrophilia is seen 4-false, hypergranular 5-true and usually asymptomatic to start with
Q15 Chronic myeloid leukemia, all are true except: 1-the absence of Philadelphia chromosome portends a poor prognosis 2-treatment with imatinib mesylate will produce 80% cytogenetic remission 3-basophilia is usually the first sign of accelerated crisis 4-associted B12 deficiency is common 5-LAP (leukocyte alkaline phosphatase) score is low
Answer 4 1-true but remember: Ph chromosome is found in 5% of childhood ALL, 25% of adult ALL and 1% of adult AML 2-true due to its inhibitory effect on the BCL-ABL fusion gene product 3-true, a useful clue 4-false, serum B12 is high due to high B12 binding protein 5-true, other causes of low LAP score: PNH and some cases of aplastic anemia and MDS
Q16 Chronic lymphocytic leukemia, all are true except : 1-trisomy 12 is the commonest cytogenetic abnormality 2-smugde cells are seen in peripheral blood film with absolute lymphocytosis of mature looking lymphocytes 3-bullous skin rash may be seen 4-monoclonal bound is seen in some patients on serum protein electrophoresis 5-hyperurecemia is common
Answer : 1-and 13q abnormalities portends poor prognosis 2-and warm immune hemolytic anemia and ITP like picture may be seen 3-true 4-as in many lymphoproliferative disorders 5-FALSE , the doubling time is usually long and the cell turnover low .
Q17 Hodgking’s disease, all are true except: 1-LDH is raised and is a useful guide to the bulk of the disease 2-usually there is anemia, neutrophilia, thrombocytosis and in some esosinophilia at the time of diagnosis 3-residual masses after treatment are common 4-lymphocyte depleted type usually has poor prognosis 5-there is an association with CMV infection
Answer 5 1-as in many hematological malignancies 2-and ESR is high and indicates an active disease 3-and hence it is difficult to know weather these represents lymphomatous masses or not, PET scan may be used to differentiate between them 4-and lymphocytic predominant has good prognosis 5-Fasle, with EBV infection
Q18 Multiple myeloma, all are true except 1-the ESR is usually very high 2-1% is non secretory 3-human herpes virus 8 infection is being increasingly recognized association 4-bone scan is usually normal 5-serum alkaline phosphatase is usually high a the time of diagnosis
Answer 5 1-true may be such a highly raised ESR in myeloma but this myeloma high ESR is responsible only for 3% of cases of ESR>100 2-keep it in mind ,on the other hand there may be no serum paraprotien but only urinary Bence John’s protein 3-true, infected stem cells may be responsible for the secretion of high level of IL-6 4-true, as there is an overall inhibitory effect on bone osteoblasts 5-false, only in the presence of a fracture
Q19 Features in favor of monoclonal gammopathy of undetermined significance (MGUS) rather than multiple myeloma (MM) are all of the followings except 1-low level of the paraprotein 2-the paraprotein remains stable over many months of period of observation 3-no bone disease 4-no renal disease 5-hypogammaglobinemia
Answer 5 Immune paresis is in favor of MM as well as bone and renal diseases
Q20 Causes of secondary polycythemia, all are true except 1-adaptation to high altitude 2-Pickwikian syndrome 3-hemoglobin M 4-primary hepatocelluar carcinoma 5-polycythemia rubra vera
Answer 5 1-and in neonates 2-and smoking, COPD and morbid obesity 3-and other high affinity hemoglobins 4-and renal cell carcinoma 5-false, a primary one.
Q21 Causes of reactive thrombocytosis, all are true except 1-post splenectomy states 2-post hemorrhage states 3-post surgical states 4-post partum states 5-polycythemia rubra vera
Answer 5 Other causes, systemic inflammatory disorders and vasculitis, trauma, infections.PRV may have a high platelet count but it is not a reactive process because it is part of the panmyelosis process seen in PRV.
Q22 Myelodysplastic syndromes, all are true except 1-seen increasingly in old people but no age is exempted 2-cytopenias are very common and dysplastic changes in the bone marrow are seen 3-up to 40 % will progress to AML 4-5q- syndrome has a relatively good prognosis and is seen mainly in woman 5-the commonest type is refractory anemia
Answer 5 1-true mainly it is a disease of elderlies 2-like hypo granular neutrophils , abnormal neutrophil nuclear lobulation , vacuolated erythroblasts 3-other causes of death are from infection and bleeding 4-and secondary MDS is seen after chemotherapy and radiotherapy 5-false the majority is refractory anemia with excess blasts in transformation
Q23 Antiphsopholipid syndrome, all are true except 1-the aPTT is prolonged and does not correct with addition of normal plasma 2-Addison’s disease is a complication 3-thrombocytopenia and immune hemolytic anemia 4-chorea, migraine and transverse myelopathy 5-there is a bleeding diathesis
Answer: 5 1-indicates the presence of inhibitors in the patient's plasma(unlike hemophilia) 2-bilateral adrenal vein thrombosis 3-may be confused with SLE per se 4-and epilepsy, TIAs, stroke and multi-infarct dementia 5-false, arterial and venous thrombosis, if hemorrage occurs it is either due to over anticoagulation or due to severe thrombocytopenia. Remember: the aPTT is already prolonged making heparin therapy follow up is difficult
Q24 Von Willibrand disease. All are true except: 1-the commonest mild inherited bleeding tendency 2-DDAVP is contraindicated in type II B 3-bleeding time is a useful screening test 4-some cases are associated with normal amount of von Willibrand factor antigen 5-the disease always has an autosomal dominant inheritance
Answer 5 1-many cases are mild and discovered later in life 2-becauses it induces further thrombocytopenia .other types have normal platelets count 3-true...The only useful indication of bleeding time application 4-but an abnormal VWf multimers 5-some cases are autosomal recessive and usually the dominant ones have a variable expression
Q25 Hemophilia A, all are true except 1-1/3rd of cases are kept in the community by a fresh new mutations 2-the PT and the bleeding time are normal 3-preferably treated by recombinant human factor 8 concentrate 4-the prolonged aPTT does correct with addition of normal plasma 5-normal activity of factor VIII is 200-300%
Answer 5 1-hence no family history 2-characteristic and the platelets may be elevated after a bleeding episode like a GIT bleeding or due to recurrent bleedings causing IDA and hence thrombosytosis 3-in severe cases should be always the first line IF AVAILABLE 4-if does not correct this indicates the development of inhibitors 5-Its activity is between 50-150%
Q26 Idiopathic thrombocytopenic purpura , all are true except : 1-the presence of splenomegaly may be a clue to chronic lymphocytic leukemia 2-in adults usually take a chronic fluctuating course rather than an acute monophasic illness 3-2/3rd responds to initial steroid therapy 4-the bone marrow has a limited role in the diagnosis 5-in adults the disease is usually preceded by an upper respiratory tract infection
Answer 5 1-and also SLE. 2-in contrast to children 3-and 2/3rd responds to splenomegally after failure of steroids 4-its role mainly to exclude other diseases like ALL in children and antiplatlets antibodies are not used routinely in the diagnosis 5-false, such a history is usually obtained in children not adults
Q27 Causes of renal impairment in multiple myeloma, all are true except : 1-hyperurecemic nephropathy 2-hypercalcemia and dehydration 3-amyloidosis and light chain deposition 4-severe and recurrent urinary tract infections and high dose NSAIDS 5-treamtent with melphalan
Answer 5 Treatment with melphalan helps to stabilize the disease and may produce improvement
Q28 Bad prognostic sings in multiple myeloma at the time of diagnosis, all are true except 1-severe hypoalbuminemia 2-plasma cell leukemia 3-low serum Beta 2 microglobulin level 4-intractable renal failure 5-hemoglobin less than 7 g/ dl
Answer 3 Also thrombocytopenia HIGH serum Beta 2 microglobulin level is a bad sign Q29 Multiple myeloma, all are true except: 1-high fluid intake is an important part of the management 2-the commonest paraprotein secreted is IgG 3-recurrent chest infections implies immune paresis rather than bone marrow failure 4-headache, blurred vision and epistaxis usually indicates hyperviscosity with paraprotinema of IgA type 5-raised serum alkaline phsophatase is common at the time of diagnosis
Answer 5 1-for many reasons, dehydration is common, 2- About 55%, IgA 21%, light chain only 22% and others including non secretory about 2% 3-true as hypogammaglobinemia is common and severe. Bone marrow failure is an advanced stage in the course of the disease. 4-true 5-indicates a recent fracture .MM per se does not raise serum alkaline phosphatase due to inhibitory cytokines on osteoblastic function and hence radio isotope scan is also normal
Q30 Hairy Cell leukemia, all are true except: 1-severe neutropenia and monocytopenia are common and characteristic 2-it is a rare indolent B cell lymphoproliferative disorder 3-the malignant B cells characteristically carry CD25 and CD103 4-the response to Cladribin is excellent with long term remission is the rule in many cases 5-lymph node enlargements are common
Answer5 1-hnece symptoms of infection are common 2-true relatively has a slow pace 3-can be used when performing flow cytometry in the diagnosis 4-and to deoxycofermycin 5-splenomegally is seen up to 90% and may be massive, but lymph node enlargement is highly unusual (unlike CLL)
Q31 Myeoldysplastic syndrome (MDS), all are true except: 1-the treatment is still unsatisfactory 2-chromosomal analysis frequently reveals abnormalities in chromosomes 15 and 17 3-the disease is probably more common than acute leukemia 4-secondary MDS mainly seen in young patients receiving chemotherapy or radiotherapy for some reason or another 5-macrocytosis is characteristic
Answer 2 1-many options are there but the treatment is mainly supportive 2-chromosomes 5 and 7 3-usually in elderly but actually in theory no age is exempted 4-like following alkylating agents or etoposide 5-with dysplastic NOT megaloblastic changes
Q32 Immune hemolytic anemia, all are true except 1-the commonest cause is the idiopathic form 2-the presence of smudge cells on blood film are useful to detect the etiology 3-direct Coomb’s test detect IgE and complement but not IgA antibodies 4-a positive direct Coomb’s test requires at least 200 antibodies to be attach to the surface of RBCs to be positive 5-there is an association with HIV infection
Answer 3 1-true and all other cases are secondary like infections, tumors, drugs 2-CLL.The presence of spherocytes per se may be a clue to AIHA but not its cause 3- Direct Coomb’s test detects IgG and complement but not IgA or IgE antibodies hence may be falsely NEGATIVE when AIHA is associated with these antibodies 4-true hence those with low tires may be falsely NEGATIVE 5-yes, also seen in ITP
Q33 Hereditary spherocytosis, all are true except 1-the commonest abnormalities are seen in beta spectrins and ankyrins 2-pigment gall stones are seen up to 50% of cases 3-recurrent severe crisis is an indication for splenectomy 4-75 % of cases are from fresh new mutations 5-Coomb’s test is negative
Answer 4 1-these are usually mild. More severe cases either indicate a coexistent defect of second different protein or coincidental polymorphism of ALPHA spectrin 2-and indicates the need for spelnectomy 3-as well as growth retardation in children and death of a family member from the disease 4-false 25% only 5-a must BUT may become positive after many transfusions i.e. alloimmunization!!
Q34 G6PD deficiency, all are true except 1-the prematurely released reticulocytes may have normal level of the enzyme 2-bite cells and blister cells are seen in peripheral blood film 3-Hienz bodies are seen only by staining the blood film with supravital stain 4-may present as neonatal jaundice 5-the African type of deficiency is very severe
Answer 5 1-hence during the acute hemolytic episode don’t assess the enzyme level as it may become transiently normal or even high 2-useful clue in acute attacks 3-so you have to give an enough HISTORY to the hematology lab so as to use this stain 4-especially after receiving the water soluble analogue of vitamin K 5-False, the Caucasian and the Oriental are the most severe when compared with the African type
Q35 Magaloblastic anemias, all are true except 1-the blood film cannot differentiate between B12 and folic acid deficiencies 2-neurological manifestations are more in favor of B12 deficiency 3-serum folate is less useful than red cell folate level 4-the response to hematinic replacement may precipitate severe hyperkalemia 5-the response to hematinic replacement is seen even within minutes
Answer 4 1-true, it just provides a clue to them like hypersegmented neutrophils 2-true, but some textbook may say peripheral neuropathy may be seen in folate deficiency also 3-true. Anorexia ,alcohol and phenytoin can severely depress the serum level in the absence of deficiency state, also a single meal may totally NORMALISE it in a severely deficient patient .So Red cell folate is better than serum folate level 4- May precipitate severe hypokalemia 5-and reticulocytes percentage may reach 50% with in the first week of treatment
Remember: Pregnancy is the commonest cause of megaloblastosis world-wide and more likely to be seen in the context of multiple pregnancy, and multiparity
Q36 Features in favor of hemolysis, all are true except 1-raised serum LDH 2-indirect hyperbilirubinemia 3-reticulocytisis 4-hemoglobinemia 5-negative urinary hemosiderin
Answer 5 Also hemosidrinuria, hemoglobinuria, reduce serum haptoglobin. Coombs test may be +ve or –ve.
Q37 Assessing iron status in Iron Deficiency Anemia (IDA), all are true except 1-ferritin is raised in acute phase responses 2-plasma iron is raised in liver disease and hemolysis 3-serum transferrin levels are low in liver disease and acute phase responses 4-transferrin saturation less than 36%is highly suggestive of iron deficiency anemia 5-increased serum soluble transferring receptors is very helpful in the diagnosis of iron deficiency anemia
Answer 4 1-but here, levels down to 100 microgram/ L is still suggestive of IDA in chronic inflammatory illnesses 2-serum iron fluctuates by 30-50% on daily basis and even diurnal basis. It is low in acute phase responses 3-and in nephritic syndrome and malnutrition .Raised in pregnancy and oral CCP 4-false, less than 16 % 5-measured by immunoassay
Q38 Severe allergic reaction associated with blood transfusions, all are true except: 1-immediately stop the infusion 2-give O2 and salbutamol nebulizer 3- Give saline wash future blood components 4-return the blood unit intact to the blood bank with all other used or unused units 5-bronchospasm, angiodema and hypotension are rare
Answer 5 Item 5 is false….they are the predominant clinical picture
Q39 Major ABO incompatibility, all are true except; 1-immediately stop the infusion, takedown the unit and the giving set and return it to the blood bank 2-commence IV saline infusion and measure urine input /output 3-inform the hospital transfusion department immediately 4-treart any DIC 5-ensure a urine out put of at least 10 ml / minute
Answer 5 Ensure urine out put of at least 100 ml / minute
Q40 Indication for a thrombophilia screen, all are true except 1-venous thrombosis in a patient younger than 65 years 2-venous thrombosis at an unusual site like portal vein 3-combined arterial and venous thrombosis 4-recurrent venous thrombosis 5-family history of venous thrombosis
Answer 1 Venous thrombosis in a patient younger than 45 years or arterial thrombosis in a patient younger than 40 years of age.
