Q6: Answer: 4 1-True, be careful as there may be a co-existent iron deficiency anemia causing a falsely low or normal HbA2. 2- Together with iron chelation therapy and daily folic acids. 3- The usually story, and then confirmed by hemoglobin electrophoresis. 4- False, may be due to severe longstanding anemia causing high out put cardiac failure. 5-True, and profound dyserythropoiesis. Q7: Answer: 5 Hemolytic disease of the new born causes mainly extra vascular hemolysis. Q8: Answer: 4 1- Any neurological syndromes + fever + skin rash =exclude TTP. 2- True for unknown reason. 3- Purpuric 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: Answer: 3 1- and eczema and other skin diseases. 2- and filiariasis, ascariasis. 3- False, treatment with steroids causes neutrophilia and eosinopenia. 4- usually forgotten. 5- PAN and esosinophilic granuloma. Q10: Answer: 5 1- and bone marrow recovery from radiotherapy. 2- and inflammatory bowel disease, SLE. 3- and myeolomonocytic leukemia. 4- and TB, typhus, brucellosis, SBE, malaria, trypanosomiasis. 5- false, causes monocytopenia. Q11: 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 neutrophilia which is a useful clue to differentiate it from viral hepatitis. Q12: Answer: 4 The common ALL type has a good prognosis. The T cell type presents with a mediastinal mass and a very high leukocyte count and has a gloomy prognosis. Also t(9, 22) cytogenetic abnormality has a very poor prognosis (unlike in CML where its presence confers a good prognosis). Q13: 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: Answer 4 1- True. 2- Retenoic Acid Receptor Alpha. 3- True, hence a rapid development of neurtrophilia is seen. 4- False, hypergranular. 5- True and usually asymptomatic to start with. Q15: 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 vitamin B12 is high due to high vitamin B12 binding protein. 5- True, other causes of low LAP score: PNH and some cases of aplastic anemia and MDS. Q16: Answer: 5 1- and 13q abnormalities, and both portend 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: 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: Answer: 5 1- true, 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 paraprotein, 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: Answer: 5 Immune paresis is in favor of MM, as well as bone and renal diseases. Q20: 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: 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: 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, refractory anemia with excess blasts in transformation. Q23: 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: 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: Answer: 5 1- hence no family history is present. 2- characteristic, and the platelets may be elevated after a bleeding episode like a GIT bleeding or due to recurrent bleedings causing iron deficiency anemia and 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: Answer: 5 1- and also SLE. 2- in contrast to children. 3- and 2/3 rd responds to splenomegally after failure of steroids. 4- its role mainly is to exclude other diseases like ALL in children, and antiplatelets antibodies are not used routinely in the diagnosis. 5- false, such a history is usually obtained in children not adults. Q27: Answer: 5 Treatment with steroids (together with melphalan) helps to stabilize the disease and may produce improvement. Q28: Answer: 3 HIGH serum Beta 2 microglobulin level is a bad prognostic sign. Q29: 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, hypogammaglobulinemia is common and severe. Bone marrow failure is an advanced stage in the course of the disease. 4- true, and may indicate the need for plasmapharesis. 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: Answer: 5 1- hence symptoms of infection are common. 2- true, relatively has a slow pace. 3- can be seen when performing flow cytometry in the diagnosis. 4- and to deoxycofermycin. 5- false, splenomegally is seen in up to 90% of cases and may be massive, but lymph node enlargement is highly unusual (unlike CLL). Q31: Answer: 2 1- many options are there but the treatment is mainly supportive. 2- false, chromosomes 5 and 7. 3- usually in elderly population, but in theory no age is exempted. 4- like following alkylating agents or etoposide. 5- with dysplastic, NOT megaloblastic changes. Q32: Answer: 3 1- true, and all other cases are secondary, like infections, tumors, drugs. 2- smudge cells are seen in 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: 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: 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- a 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 types are the most severe when compared with the African type. Q35: Answer: 4 1- true, it just provides a clue to them like hypersegmented neutrophils 2- true, but (written in some textbooks) a peripheral neuropathy state 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- false, 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: Answer: 5 Hemosidrinuria, hemoglobinuria, and reduced serum haptoglobin all are seen. Q37: Answer: 4 1- and levels down to 100 microgram/ L are 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 nephrotic syndrome and malnutrition .It is raised in pregnancy and oral CCP. 4- false, less than 16 %. 5- measured by immunoassay. Q38: Answer: 5 They (bronchospasm, angiodema and hypotension) predominate the clinical picture. Q39: Answer: 5 Ensure urine out put of at least 100 ml / minute. Q40: Answer: 1 Item 1 is wrong because he has a clear cut precipitating factor, besides this is the first episode, so no need to screen. Chapter X / Rheumatology Answers Q1: Answer: 3 1-true, is an autoantibody. 2-but, we detect only IgM in the slide agglutination test. 3-false, does not reflect any thing apart from poor prognosis if found in high titer in RA. 4- like slide tube agglutination, SCAT and Rose Waaler tests. 5-true, must be seropositive here. Q2: Answer: 5 1- as it rises rapidly and falls rapidly and hence reflects the degree of the APRs. 2- true, as it rapidly rises from a low to high levels in acute inflammatory process and closely mirrors the degree of inflammatory. 3- true, in cases of improvement in the APR, the CRP normalizes rapidly while the ESR LAGS behind for a variable period of time. 4- and in polycythemia rubra vera (PRV), so in these cases we measure the plasma viscosity as a measure of APR (remember in PRV there is florid APR; yet the ESR is very low). 5- the CRP is elevated in the presence of infection of trauma in SLE, other wise in acute flare ups it is normal;a useful CLUE! Q3: Answer: 4 1-hypochromic microcytic one is seen up to 25 % but does not respond to iron therapy. It is not an iron deficiency states, but there is defects in ferro-kinetics and iron utilization; unfortunately may be complicated by true iron deficiency anemia eg long term treatment with NSAIDS. 2- true, useful guide to the overall activity eg in RA. 3-true, varieties of pictures, and may be complicated by drug side effects on the blood count and bone marrow ,so keep it in mind. 4-leukocytosis and neutrophilia eg in classical PAN. 5-true, a common cause of neutrophila. Q4: Answer: 4 1- eg using rodent organs or human cell lines. 2- like rheumatoid factor, low tires may be seen in healthy normal people in a good percentage. 3-hence its name. 4- FALSE, 100% of cases; a negative titer virtually excludes drug induced lupus (here it is mainly anti histone H2 A and B subtypes). 5- depends on the antigen preparation used in the test and whether we detect IgG or IgM type; however the tests are unfortunately NOT standardized and liaison with local labs is important. Q5: Answer: 5 1- with a fall from standing height or less. 2- also radiological evidence of osteoporosis, and premature gonadal failure. 3-remmeber, not only the disease may pose a risk but also its treatment like corticosteroids. 4-also, clinical features of osteoporosis, like loss of height and kyphosis. 5-false ,BMI less than 19; hence obese persons are usually protected. Remember: polycystic ovarian syndrome patients although may be infertile with irregular or absent cycles, but the high BMI and the good amount of estrogens and androgens protect them from osteoporosis. Q6: Answer: 5 1- as the isotop is taken by body bones. 2- also in primary and secondary bone tumors. 3- as the Tc-biphosphanate later localizes to areas of bone remodeling; theses LATE images are usually taken after few hours. 4-true, the main indication in general. 5-false, it depends on the osteoblatstic activity which is inhibited in multiple myeloma; so it is not useful here, but only if it was complicated by fractures. Remember: although bone scan has a high sensitivity, it extremely lacks specificity and lacks high anatomical resolution. Q7: Answer: 5 Other indications: reflex sympathetic dystrophy and hypertrophic osteoarthropathy; and useful in the assessment of the extent of Paget’s disease of the bone. In multiple myeloma (MM), it depends on the osteoblatstic activity which is inhibited in MM, so it is not useful here, only if it was complicated by fractures. Q8: Answer: 1 1- false, less than 20 and more than 50 years of age. 2- and a history of a major trauma. 3- and the presence of a painful spinal deformity, severe symmetrical spinal deformity, saddle anesthesia, progressive neurological signs in the lower limbs, sphincter dysfunction, and a sensory level 4- very important indicators. 5- and HIV, malignancy, on long term steroids etc. Q9: Answer: 5 90% of cases improve after 6 weeks so it has an excellent prognosis. Other features: Tendency for recurrent episodes, pain is limited in the back or thigh but NEVER below the knee, and no clear cut root signs. Q10: Answer: 5 In general, the prognosis is reasonable with 50% recovery at 6 weeks. Q11: Answer: 4 1- so it is common. 2- true associations, also hyperinsulinemia. 3- true, at least 4. 4- FALSE, there is NO such features which are (with posterior apophyseal joint involvement) indicate spinal spondylosis. 5-true, very rarely have symptoms, usually low back pain. Q12: Answer: 5 1- and from capsular fibrosis and intervening large osteophytes; There is palpable, sometimes, audible coarse crepitus. 2- so called "good days and bad days". 3- also may increase upon joint movement. 4- usually one or few painful joints (rarely multiple painful areas). 5- false, NO such joint instabilities Q13: Answer: 5 1- ogether with trauma; usually are monoarticular. 2- like JIA . 3- and the so called endemic osteoarthritis. 4- and hemachromatosis. 5- FALSE, arthropathy is seen up to 50% of cases of acromegally (not in GH deficiency). Q14: Answer: 5 1- true, you should treat appropriately to minimize disability. 2- true, they are “safe and cheap”. 3- true, with excellent results. 4- true, with no significant drug-drug interaction and are often effective at relieving pain. 5- false, a common age associated phenomenon, and may precipitate acute pseudogout attacks. Q15: Answer: 4 In Caucasians, the prevalence is about 1-1.5 % with female to male ratio of 3:1 Q16: Answer: 1 HLA DR4 is the major susceptibility haplotype in most ethnic group eg found in 75% of Caucasian patients with RA, and HLA DR4 positivity is more common in those with severe disease. HLA DR1 is more important in Indians and Israelis while HLA DW15 is more important in Japanese. Q17: Answer: 2 1- true, with infiltrations by lymphocytes, plasma cells and macrophages. 2- CD4 positive cells. 3- similar granulomatous lesions are seen in the sclera and pericardium, pleura and lungs. 4- fibrous or bony ankylosis occurs late. 5- the regional lymph nodes draining the actively inflamed joints are frequently hyperplasic. Q18: Answer: 5 1- and when rupture may simulate DVT, but remember both may coexist together as the patient may be immobile and there is an increased risk of DVT, so be careful!! 2- usually seen in extensor body surfaces, sclera and lung. 3- Episcleritis is a benign complication but sclertits is serious. 4- seen in up to 30% of nodular seropositive patients, but constrictive pericarditis is rare. 5- the CNS is surprisingly SPARED in RA vasculitis. Q19: Answer: 1 1- false, less than 1 % only. 2- and nodular seropositive disease. 3- and weight loss, recurrent infections, and sicca syndrome. 4- and normochromic anemia, defective T and B cell function and neutropenia. 5- usually between 50-70 years of age. Q20: Answer: 4 1- mouth ulceration and Pemphigus are both rare. 2- drug induced lupus and Goodpasture's syndrome are also rare. 3- rapidly falling platelet count, mild thrombocytopenia, and proteinuria are indications to stop the drug and reintroduce it slowly, but if they recur then stop it for good. 4- febrile reactions and pancytopenia are absolute indication to stop the drug IMMEDIATELY and for GOOD. 5-and glomerulonephritis. Q21: Answer: 3 Marrow suppression and aplastic anemia may occur and both carry a significant mortality. Q22: Answer: 2 1- an association with HLA B27. 2- peripheral asymmetrical oligoarthritis affecting the lower limbs more than the upper. [...]... Answer: 3 1- occurs in 25% and conjunctivitis in 20% 2- although extra spinal involvement is seen up to 40 % 3- false, up to 1% only; also aortitis, aortic and mitral regurgitations and cardiac conduction defects 4- because there will be loss of hip flexion which can not compensate for the spinal rigidity 5- true and the patients should receive bisphosphanates Q 25: Answer: 4 Male to female ratio is 15: 1 May... hypophophatasia Q 34: Answer: 5 Tophi are the hallmark of prolonged hyperuricemia, not pseudogout, although both may coexist! Q 35: Answer: 5 1- true, it is not a cold case 2- true, with appreciable morbidity 3- inpatient management, not on an outpatient basis 4- but the culture is positive in only 25% of gonococcal cases, while the genital tract cultures are positive in 70% in these cases! 5- false, staph... staph aureus Q36: Answers: 4 1- true, age and female genders are risk factors 2- no age is an exempt, but it s rare in young people 3- only 2 consistent associations had been shown: sleep abnormality and abnormal pain perception 4- false, the ESR should be normal 5- true , this is an evidence based intervention Q37: Answer: 5 Tibolon is a hormone receptor modulator that acts as a partial agonist at... parenchymal involvement Q48: Answer: 5 1- true, and other features reflect systemic involvement like MI, stroke, and renal failure 2- sometimes esosinophilia, like in Church Strauss vasculitits 3- true, and the treatment is mainly targeted against the hepatitis B infection 4- and sometimes methotrexate is used 5- cANCA is found up to 90% of active Wegner’s granulomatosis Q49: Answer: 4 Giant cell arteritis... rare now a days 2- a congenital defect in bone specific alkaline phosphatase 3- true, no need for calcium at all 4- false, type I responds well to active vitamin D metabolites; type II is very resistant (together with hypophsphatasia, are both the most difficult to treat) 5- a true diagnosis by bone biopsy Q41: Answer: 5 1- indicates that the bone targeting insult occurs early in life 2- causing bone... lesions 2- but contractures may occur due to tendon inflammation and may be seen as bone deformity (Jaccoud's arthropathy) 3- true, portends a poor prognosis and should be treated aggressively 4- the effusions are exudative; rarely chest pain is due to MI 5- false, mild neuropsychiatric manifestations (like depression or psychosis) and seizures Q 44: Answer: 2 Erosive arthropathy is highly uncommon Q 45: ... 3- but there is association with enthesopathy 4- true 5- like iritis (up to 2 0-2 5% ), and aortitis (4% ) which are the commonest extra particular manifestations Q23: Answer: 2 The peak incidence is in the 2nd and 3rd decades The incidence is higher in Pima and Haida Indians because of higher prevalence of HLA B27 The Kelbsiella carriage may be responsible for joint and eyes disease flares ups Q 24: Answer:... like HGPRTase deficiency Q32: Answer: 5 1- the usual story in those on thiazides of more than 18 months duration 2- true and the hands, not the feet, are the usual targets in those patients 3- as 100mg / day 4- true, followed by early mobilization 5- NSAIDs and Colchicine are best avoided because of increased incidence and severity of toxicities Q33: Answer: 3 Hyper- not hypoparathyroidism Other causes:... abnormal architecture and reduced mechanical strength 3- in the majority of patients 4- usually in those with limited cardiac reserve 5- osteosarcoma is RARE and portends a very POOR prognosis and should be suspected in any rapid increase in pain or swelling of an affected bone Q42: Answer: 1 There is polyclonal B and T cell activation Q43: Answer: 5 1- also there may be discoid rash, photosensitive rash,... balanitis is seen in up to 2 0 -5 0% of cases, and are usually painless and easily escape notice Q27: Answer: 2 Synchronous onset of skin disease and joint disease is uncommon and seen in up to 5% of cases Q28: Answer: 2 1- notice that arthritis mutilans is seen in 5% of cases only 2- FALSE; they are found more commonly in psoriatic arthritis ( 85% ) than in uncomplicated psoriasis (30%) 3- true, like genitals, . 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 Q 14: Answer 4 1- True. 2- Retenoic Acid Receptor Alpha. 3- True, hence a. AVAILABLE. 4- if does not correct this indicates the development of inhibitors. 5- its activity is between 5 0-1 50 %. Q26: Answer: 5 1- and also SLE. 2- in contrast to children. 3- and 2/3 rd . neurtrophilia is seen. 4- False, hypergranular. 5- True and usually asymptomatic to start with. Q 15: Answer: 4 1- True, but remember; Ph chromosome is found in 5% of childhood ALL, 25% of adult ALL