path haematology - formatted

Question 1

1. 92.APRIL02 Concerning Hodgkin’s disease, which of the following statements IS INCORRECT?
2. Nodular sclerosing sub-type has an excellent prognosis
3. Lymphocyte depleion sub-type has a poor prognosis
4. Long term survivors of chemotherapy and radiotherapy are of increased risk of developing lung cancer
5. Pulmonary involvement occurs by extension of disease from hilar nodes to peri-bronchovascular interstitial tissue
6. Involvement of mesenteric nodes and Waldeyer’s ring is common

Answer 1

5. Involvement of mesenteric nodes and Waldeyer’s ring is common (uncommon)
6. 92.APRIL02 Concerning Hodgkin’s disease, which of the following statements IS INCORRECT? (–)
7. Nodular sclerosing sub-type has an excellent prognosis (Most common form (65 – 75% cases) and has excellent prognosis)
8. Lymphocyte depletion sub-type has a poor prognosis (True but controversial entity)
9. Long term survivors of chemotherapy and radiotherapy are of increased risk of developing lung cancer ( Myelodysplastic syndromes, AML, lung, stomach, melanoma, breast (esp young women treated with radiotherapy to chest), NHL)
10. Pulmonary involvement occurs by extension of disease from hilar nodes to peri-bronchovascular interstitial tissue (pulmonary involvement usually extends from hilum in untreated Hodgkins)
11. Involvement of mesenteric nodes and Waldeyer’s ring is common (uncommon)

Question 2

2. 93.APRIL02 Which of the following statements concerning Langerhan’s Histiocytosis is correct?
3. The term encompasses the older designations Hand-Schuller-Christian disease and Eosinophilic Granuloma. Letterer-Siwe now considered a separate condition.
4. In those under two years of age, bony lesions dominate the disease
5. The bones most commonly involved include the pelvis, scapula and clavicles
6. The diagnosis cannot be made without histology confirming Birbeck granules
7. It is associated with recurrent ear and respiratory infections

Answer 2

5. It is associated with recurrent ear and respiratory infections – T - Multifocal Langerhans cell histiocytosis usually affects children, who present with fever; diffuse eruptions, particularly on the scalp and in the ear canals; and frequent bouts of otitis media, mastoiditis, and upper respiratory tract infections.
6. 93.APRIL02 Which of the following statements concerning Langerhan’s Histiocytosis is correct? (–)
7. The term encompasses the older designations Hand-Schuller-Christian disease and Eosinophilic Granuloma. Letterer-Siwe now considered a separate condition – F - In the past, these disorders were referred to as histiocytosis X and subdivided into three categories: Letterer-Siwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma. These three conditions are now believed to represent different expressions of the same basic disorder
8. In those under two years of age, bony lesions dominate the disease – F - The dominant clinical feature is the development of cutaneous lesions that resemble a seborrheic eruption secondary to infiltrations of Langerhans histiocytes over the front and back of the trunk and on the scalp.
9. The bones most commonly involved include the pelvis, scapula and clavicles – F - Virtually any bone in the skeletal system may be involved, most commonly the calvaria, ribs, and femur
10. The diagnosis cannot be made without histology confirming Birbeck granules – F - Because Birbeck granules are not seen in all tumor cells by electron microscopy, the detection of CD1a expression by immunohistochemical techniques aids in the diagnosis
11. It is associated with recurrent ear and respiratory infections – T - Multifocal Langerhans cell histiocytosis usually affects children, who present with fever; diffuse eruptions, particularly on the scalp and in the ear canals; and frequent bouts of otitis media, mastoiditis, and upper respiratory tract infections.
    • Acute disseminated Langerhans cell histiocytosis ( Letterer-Siwe disease) occurs most frequently before 2 years of age but occasionally may affect adults.
    • Most of those affected have concurrent hepatosplenomegaly, lymphadenopathy, pulmonary lesions, and eventually destructive osteolytic bone lesions.

Question 3

3. 94.APRIL02 You are following up a patient whose skull x-ray you reported as suspicious of myeloma. The hospital pathology computer shows that the serum electrophoretic analysis and urinary Bence Jones protein assessment are normal. Which of the following statements is most correct?
4. These finding excludes multiple myeloma
5. Approximately 1 in 100 cases of multiple myeloma would fit this pattern.
6. Approximately 20% of cases of multiple myeloma would fit this pattern.
7. Bence Jones protein assessment should have done on serum; in approximately 70% of patients the electrophoretic pattern alone is normal.
8. These tests are not relevant to the diagnosis of multiple myeloma

Answer 3

2. Approximately 1 in 100 cases of multiple myeloma would fit this pattern – T - probably most true. Nonsecretory MM in ~3%. (GC: 1%, Robbins)
3. 94.APRIL02 You are following up a patient whose skull x-ray you reported as suspicious of myeloma. The hospital pathology computer shows that the serum electrophoretic analysis and urinary Bence Jones protein assessment are normal. Which of the following statements is most correct? (TW) (–)
4. These finding excludes multiple myeloma – F - the absence of paraproteinemia and paraproteinuria does not exclude myeloma
5. Approximately 1 in 100 cases of multiple myeloma would fit this pattern – T - probably most true. Nonsecretory MM in ~3%. (GC: 1%, Robbins)
6. Approximately 20% of cases of multiple myeloma would fit this pattern – F - nonsecretory MM 3%.
7. Bence Jones protein assessment should have been done on serum; in approximately 70% of patients the electrophoretic pattern alone is normal – F - in 16% of patients with MM, only a Bence Jones protein (light chain) was produced, often too low to be detected by routine immunofixation techniques. These tend to be concentrated in urine.
8. These tests are not relevant to the diagnosis of multiple myeloma – F - the most important diagnostic finding in MM is the demonstration of a monoclonal protein, which is present in the serum and/or urine in 97% of patients.
   Multiple myeloma is characterized by the neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin.
   Approximately 3% of patients with MM have no M-protein in the serum or urine on immunofixation at the time of Dx and are considered to have nonsecretory myeloma. The condition remains nonsecretory in most patients (76%).
   • The monoclonal Ig produces a high spike when serum or urine is subjected to electrophoresis.
   • Immunoelectrophoresis or immunofixation is used to identify the nature of monoclonal Ig
   • Quantitative analyses of monoclonal Ig usually reveal more than 3 gm of Ig per dl of serum and more than 6 mg of Bence Jones proteins per dl of urine.
   • The most common serum monoclonal Ig (M protein) is IgG, which is found in 55% of patients.
   • An additional 25% of cases are associated with an IgA M protein.
   • Myelomas expressing IgM, IgD, or IgE occur but are rare.
   • Excessive production and aggregation of M proteins leads to the hyperviscosity syndrome (described under lymphoplasmacytic lymphoma) in approximately 7% of patients, most of whom have tumors that secrete IgA or IgG3 .
   • Bence Jones proteinuria and a serum M protein are both observed in 60 to 70% of all myeloma patients.
   • In approximately 20% of patients, however, Bence Jones proteinuria is present as a isolated finding.
   • It should also be noted that about 1% of myelomas are nonsecretory and thus occur in the absence of detectable serum or urine M proteins.
   o Hence, the absence of paraproteinemia and paraproteinuria does not exclude myeloma

Question 4

4. 95.APRIL02 A 48 year old man is referred for a CT scan from his gastroenterologist for a gastric marginal zone lymphoma / MALToma. Which of the following statements concerning marginal zone lymphomas IS LEAST correct?
5. They are a form of B cell lymphoma
6. They are most common in middle aged adults
7. Early dissemination is typical
8. They are associated with Helicobacter gastritis
9. They are associated with chronic inflammation secondary to autoimmune disorders

Answer 4

3. Early dissemination is typical (tend to remain localized for long period)
4. 95.APRIL02 A 48 year old man is referred for a CT scan from his gastroenterologist for a gastric marginal zone lymphoma / MALToma. Which of the following statements concerning marginal zone lymphomas IS LEAST correct? (TW) (–)
5. They are a form of B cell lymphoma – T – NHL subtype of marginal zone lymphoma includes 3 distinct diseases (appear to arise from post-germinal center marginal zone B cells): Splenic MZ B-cell lymphoma; Extranodal MZ B-cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma); and Nodal MZ b-cell lymphoma.
6. They are most common in middle aged adults - T
7. Early dissemination is typical (tend to remain localized for long period)
8. They are associated with Helicobacter gastritis – T – related to chronic immune stimulation. H. pylori infection / chronic gastritis association and the development of gastric MALT is the prototypical example.
9. They are associated with chronic inflammation secondary to autoimmune disorders – T – see ans d.
   Arise within tissues affected by chronic inflammation
   • salivary gland in Sjögren disease
   • thyroid in Hashimoto thyroiditis
   • stomach in H pylori gastritis

Question 5

5. 96.APRIL02 Which of the following statements concerning small lymphocytic lymphoma IS LEAST correct?
6. It differs from chronic lymphocytic leukaemia only in the extent of peripheral lymphocytosis
7. The patients are frequently asymptomatic
8. Generalised Ivmphadenopathy and hepatosplenomegaly are seen in over half the patients
9. Approximately 10-20% of cases transform to a more aggressive lyphmoid neoplasm
10. Prognosis is poor with a 10% 5 year survival

Answer 5

5. Prognosis is poor with a 10% 5 year survival – F - Median survival 4 – 6 yrs
6. 96.APRIL02 Which of the following statements concerning small lymphocytic lymphoma IS LEAST correct? (TW) (–)
7. It differs from chronic lymphocytic leukaemia only in the extent of peripheral lymphocytosis – T – according to the WHO classification, B-cell small lymphocytic lymphoma is considered to be identical to the mature B-cell neoplasm chronic lymphocytic leukemia.
8. The patients are frequently asymptomatic – T – patients usually seek medical attention for painless generalized lymphadenopathy, which has frequently been present for several years.
9. Generalised lymphadenopathy and hepatosplenomegaly are seen in over half the patients - T
10. Approximately 10-20% of cases transform to a more aggressive lymphoid neoplasm - F - Richter’s transformation is the development of an aggressive large-cell lymphoma in the setting of underlying chronic lymphocytic leukemia / small lymphocytic lymphoma. Incidence estimated at 2-9%.
11. Prognosis is poor with a 10% 5 year survival – F - Median survival 4 – 6 yrs

Question 6

7. 97.APRIL02 Which of the following statements concerning follicular lymphoma IS LEAST correct?
8. It is a B cell lymphoma
9. While rare in Asia, it is the most common form of non-Hodgkin’s lymphoma in the United States
10. Transformation to a more aggressive lymphoma occurs in 30 to 50% cases
11. Untreated prognosis is poor (10% 5 year survival) but improves to 60-70 % with aggressive chemotherapy/ bone marrow transplant
12. Involvement of extra-nodal sites is uncommon.

Answer 6

4. Untreated prognosis is poor (10% 5 year survival) but improves to 60-70 % with aggressive chemotherapy/ bone marrow transplant – F - Although follicular lymphoma is incurable, it often follows an indolent waxing and waning course. The overall median survival is 7 to 9 years and is not improved by aggressive therapy; hence, the usual clinical approach is to palliate patients with low-dose chemotherapy or radiation when they become symptomatic.
5. 97.APRIL02 Which of the following statements concerning follicular lymphoma IS LEAST correct? (–) (TW)
6. It is a B cell lymphoma – T – defined a lymphoma of follicle center B-cells, which has at least a partially follicular pattern.
7. While rare in Asia, it is the most common form of non-Hodgkin’s lymphoma in the United States – T (at the time) – FL is the most common of the indolent NHLs. (UTD) Second most common lymphoma in the US and western Europe (grade I subtype is the most common). FL is less common in Asians and blacks.
8. Transformation to a more aggressive lymphoma occurs in 30 to 50% cases – T - Transformation occurs to diffuse large B-cell lymphoma in 30 – 50%, rarely to aggressive form of lymphoblastic lymphoma or leukaemia
9. Untreated prognosis is poor (10% 5 year survival) but improves to 60-70 % with aggressive chemotherapy/ bone marrow transplant – F - Although follicular lymphoma is incurable, it often follows an indolent waxing and waning course. The overall median survival is 7 to 9 years and is not improved by aggressive therapy; hence, the usual clinical approach is to palliate patients with low-dose chemotherapy or radiation when they become symptomatic.
10. Involvement of extra-nodal sites is uncommon – T - Extranodal site involvement (i.e. GIT, CNS, testis) uncommon
    • Follicular lymphoma is the most common form of NHL in the United States, comprising about 45% of adult lymphomas.
    • It usually presents in middle age and afflicts men and women equally.
    • It is less common in Europe and rare in Asian populations

Question 7

8. 99.APRIL02 A 24-year-old woman with Stage II lymphocyte predominate Hodgkin’s lymphoma has been neutropaenic for 2 weeks in intensive care. Since her pre-treatment CT scan 3 months earlier her liver and spleen have increased in size and show new lesions up to 2 cm in size. Despite multiple antibiotics-she continues to decline and CT shows a new left MCA infarct. This is most compatible with;
9. Systemic candidiasis
10. Bacterial endocarditis with systemic emboli /infarction
11. Drug resistant lymphocyte predominate Hodgkin’s lymphoma
12. Transformation to a more aggressive lymphoma
13. Non-bacteria thrombotic endocarditis with systemic emboli / infarction

Answer 7

5. Non-bacteria thrombotic endocarditis with systemic emboli / infarction - T

• *LJS - unsure. Marrantic endocarditis typically in mucinous adenoca. Though this would fit with decline despite abx. Systemic candidiasis also possible - causes mycotic anerysms and thrombosis causing infarcts. Also causes liver and spleen lesions, though typically small lesions
  https: //radiopaedia.org/articles/neurocandidiasis?lang=gb

8. 99.APRIL02 A 24-year-old woman with Stage II lymphocyte predominate Hodgkin’s lymphoma has been neutropaenic for 2 weeks in intensive care. Since her pre-treatment CT scan 3 months earlier her liver and spleen have increased in size and show new lesions up to 2 cm in size. Despite multiple antibiotics-she continues to decline and CT shows a new left MCA infarct. This is most compatible with: (–)
9. Systemic candidiasis could be this, with vasculitis causing the infarct
10. Bacterial endocarditis with systemic emboli /infarction
11. Drug resistant lymphocyte predominate Hodgkin’s lymphoma
12. Transformation to a more aggressive lymphoma
13. Non-bacteria thrombotic endocarditis with systemic emboli / infarction - T
    • LP Hodgkins usually has a good prognosis
    • Stage II = Involvement of two or more lymph node regions on the same side of the diaphragm alone (II) or with involvement of limited contiguous extralymphatic organ or tissue (IIE )
    • in contrast to other forms of HD, approximately 3 to 5% of tumors transform to diffuse large cell lymphomas, which are uniformly of B-cell type
    o Patients with diffuse large B-cell lymphoma typically present with a rapidly enlarging, often symptomatic mass at a single nodal or extranodal site.
    o Involvement of the gastrointestinal tract, skin, bone, or brain may be the presenting feature.
    o Waldeyer ring, the oropharyngeal lymphoid tissues that include the tonsils and adenoids, is also commonly involved. There may be large destructive masses in the liver and spleen
    o Bone marrow involvement occurs late in the disease, and rarely a leukemic picture may emerge

Question 8

9. Sep03.11 Hodgkin’s disease, IIB. Which is the most correct answer?
10. unexplained fever, night sweats or unexplained weight loss >10%
11. liver involvement

Answer 8

*LW:
Option 1: unexplained fever, night sweats or unexplained weight loss >10%, which is referring to (B) designation.
Liver involvement is stage IV disease.

All stages are further divided into
(A) Absence of B type symptoms, or
(B) Presence of B type symptoms of fever, night sweats, weight loss.

I: involvement of single lymph node region (I), or involvement of single extralymphatic organ (IE)

II: Involvement of two or more lymph node regions on the same side of the diaphragm alone (II) or with involvement of limited contiguous extralymphatic organ or tissue (IIE )

III: Involvement of lymph node regions on both sides of diaphragm (III) which may include spleen (IIIS), limited contiguous extralymphatic organ or site (IIIE), or both (IIIES)

IV: Multiple or disseminated foci of involvement of one or more extra lymphatic organs or tissues with or without lymphatic involvement.

Question 9

10. 40.APRIL02 A 32 year-old woman in the third trimeter of her first pregnancy develops HELP syndrome. This is
11. Hemolysis elevated liver enzymes and low platelets
12. Hepatic eosinophilia with low level of prothrombin
13. Haemolytic eosinophilic liver necrosis with low platelets
14. Hemorrhagic encephalopathy/Ieukomaiacia with low platelets
15. Hemorrhagic encephalopathy and leukocyte lysis with platelet autolysis

Answer 9

1. Hemolysis elevated liver enzymes and low platelets
2. 40.APRIL02 A 32 year-old woman in the third trimester of her first pregnancy develops HELP syndrome. This is: (–)
3. Hemolysis elevated liver enzymes and low platelets
4. Hepatic eosinophilia with low level of prothrombin
5. Haemolytic eosinophilic liver necrosis with low platelets
6. Hemorrhagic encephalopathy/Ieukomaiacia with low platelets
7. Hemorrhagic encephalopathy and leukocyte lysis with platelet autolysis

• Hemolysis, Elevated Liver enzymes and low Platelet count - complicating Preeclampsia-eclampsia
• Preeclampsia is a complication of pregnancy and is characterized by maternal hypertension, proteinuria, peripheral edema, coagulation abnormalities, and varying degrees of disseminated intravascular coagulation .
• When hyperreflexia and convulsions occur, the condition is called eclampsia. Hepatic disease is distressingly common in preeclampsia, usually as part of a syndrome of hemolysis, elevated liver enzymes, and low platelets, dubbed the HELLP syndrome.

Question 10

11. 12.02.18 Findings on ultrasound on 16year old female with spherocytosis, which is least likely ?
12. Gall stones
13. Renal stones
14. Splenomegaly
15. Enlarged lymph nodes

Answer 10

2. Renal stones - F
3. 12.02.18 Findings on ultrasound on 16year old female with spherocytosis, which is least likely ? Rob p398 (–) (GC)
4. Gallstones - T - (bilirubin-rich stones may occur in extravascular haemolysis – ie. pigment stones , occurs 40-50%)
5. Renal stones - F
6. Splenomegaly - T - (occurs in most form of haemolytic anemias as there is active hyperplasia of the mononuclear system. Splenomegaly occurs more often and is more marked in HS than other haemolytic anaemias)
7. Enlarged lymph nodes - T - (extramedullary hematopoiesis), theoretically.

HS: autosomal dominant disorder caused by inherited mutations that affect RBC membrane skeleton, leading to loss of membrane and eventual conversion of RBCs to spherocytes, which are phagocytosed and removed in the spleen Manifested by anaemia, splenomegaly and jaundice. Aplastic crises may be triggered by parvovirus B19 infection.
(GC) Changed Qu from “What are the likely findings”
(–) ? question should be ‘A child with spherocytosis has abdominal pain best explained by’; answer would then be Gallstones. If question asked ‘which of the following would not be seen’ then it would be ?renal stones.

Question 11

12. 12.03.30 In Thalassemia Major which is TRUE?
13. Anaemia is secondary to abnormal β-haemoglobin
14. Present at age 6 to 9 months
15. Splenomegaly only occurs if cirrhosis

Answer 11

2. Present at age 6 to 9 months - T - (change over from HbF to HbA)
3. 12.03.30 In Thalassemia Major which is TRUE? Rob p403 (–) (GC)
4. Anaemia is secondary to abnormal β-haemoglobin - F - see below
5. Present at age 6 to 9 months - T - (change over from HbF to HbA)
6. Splenomegaly only occurs if cirrhosis - F - (extramedullary haematopoiesis & increased RES function)
   Thalassaemias are a group of autosoomal codominant disorders in which mutations in the alpha or β globin genes result in reduced Hb synthesis, causing a microcytic hypochromic anaemia. β-thalassaemia major occurs when any two B0 and B+ alleles are inherited (homozygous or compound heterozygous), whilst β-thal minor occurs when only one abnormal allele is inherited. Most common cause is mutations that lead to aberrant mRNA processing.
   • β+ results in reduced β globin synthesis
   • β0 results in total β globin absence
   (ie. doesn’t form abnormal β globin)

Question 12

13. 12.02.63 Elderly man with polycythaemia. Non-associated finding on CT is:
14. Emphysema
15. Renal mass
16. Chronic pancreatitis
17. Splenomegaly

Answer 12

3. Chronic pancreatitis
4. 12.02.63 Elderly man with polycythemia. Non-associated finding on CT is: (–) (GC)
5. Emphysema - T - (any lung disease)
6. Renal mass - T - (renal cell carcinoma)
7. Chronic pancreatitis
8. Splenomegaly - T - (slightly enlarged secondary to extramedullary haematopoiesis and vascular congestion, in 75%)
   Polycythemia - relative (haemoconcentration) or absolute (primary or secondary)
   Primary: autonomous prolifn of myeloid stem cells, nearly all have mutation in JAK2 (tyrosine kinase), RBC is rendered hypersensitive to EPO (normal or low EPO levels), occurs in 40-60yo.
   Secondary (increased erythropoietin levels):
   • Appropriate:
   o Lung disease, high altitude living, cyanotic heart disease
   • Inappropriate:
   o Erythropoitetin-secreting tumours
    Renal cell carcinoma
    Hepatoma
    Cerebellar haemangioblastoma
    Uterine Fibroids
   o Surreptitious erythropoieten use (athletes)

Question 13

14. 12.03.01 Patient with suspected mononucleosis, which would be ATYPICAL?
15. Abnormal LFT’s
16. Proteinuria with viral inclusion in epithelial cells in urine
17. Groin lymphadenopathy
18. Mononuclear meningitis
19. Atypical lymphocytes in FNA

Answer 13

2. Proteinuria with viral inclusion in epithelial cells in urine - F
3. 12.03.01 Patient with suspected mononucleosis, which would be ATYPICAL ? Rob p417-418 (–) (GC)
4. Abnormal LFT’s - T - liver fxn is almost always transiently impaired to some degree. Assocd with jaundice, elevated enzymes, disturbed appetite, and rarely even liver failure.
5. Proteinuria with viral inclusion in epithelial cells in urine - F
6. Groin lymphadenopathy - T - widespread lymphadenopathy with atypical lymphocytes
7. Mononuclear meningitis - T - occurs but is rare
8. Atypical lymphocytes in FNA - T - atypical lymphocytes occur in both peripheral blood smears and lymph nodes FNA. Mainly cytotoxic T-cells; occasionally cells resembling Reed Sternberg cells are present.
   Note : Infectious mononucleosis
   • herpes virus family
   • associated with the development of Burkitt lymphoma and nasopharyngeal carcinoma
   • splenomegaly occurs

Question 14

15. 12.03.35 Solitary plasmocytoma of bones of nasal cavity. Which is TRUE?
16. Must go onto MM
17. 20% progress to multiple myeloma
18. mucosal covered
19. <2cm bone expansion

Answer 14

2. 20% progress to multiple myeloma - T - rate of progression to MM is lower than in solitary bone plasmactyoma, ranging from 11-30% at 10yrs (eMed). Som says eventually 35-50% with primary extramedullary plasmacytoma develop MM.
3. 12.03.35 Solitary plasmacytoma of nasal cavity. Which is TRUE ? (GC)
4. Must go onto MM - F - not all progress, see below.
5. 20% progress to multiple myeloma - T - rate of progression to MM is lower than in solitary bone plasmactyoma, ranging from 11-30% at 10yrs (eMed). Som says eventually 35-50% with primary extramedullary plasmacytoma develop MM.
6. mucosal covered - F - histologically a monoclonal infiltration of plasma cells, commonly exhibits submucosal growth; low, intermed or high grade.
7. <2cm - F - mass of one to several centimetres, well-defined lobulated border.
8. bone expansion - ?? - homogeneous enhancing polypoid masses that remodel surrounding bone [Som]; eMedicine says EMP causes local bone destruction in most pts with nasal cavity or maxillary sinus involvement.

Solitary plasmacytoma:
• may be skeletal (solitary bone P) or soft tissue (extramedullary) in origin
• SBP occurs in a similar distribution to MM (predilection for red marrow of axial skeleton); classically causes bone destruction; rarely a cystic lesion, or slcerotic lesion (latter assocd with POEMS syndrome).
• EMP occurs mainly in aerodigestive tract (80% in the head & neck) - 28% in nasal cavity, 22% in paranasal sinuses. Around 30-75% of pts with EMP are alive after 5yrs, however, the median length of survival after onset of MM is <2yrs. Treatment is with surgery and radiotherapy.
[Som, eMedicine, Robbins]

Question 15

17. 2.03.34 Patient with Waldenstroms macroglobulinaemia, which is most correct ?
18. Bone lesions indistinguishable from multiple myeloma
19. Bone lesions in <3%
20. Bone lesions involving cortex
21. Solitary Bone lesion most likely similar sites as multiple myeloma but spares skull
22. Subperiosteal deposits – endosteal spread

Answer 15

2. Bone lesions in <3% - T - unlike myeloma, lytic bone lesions are “uncommon” (eMed). Robbins says “does not produce lytic bone lesions”; Dahnert says “lytic lesions seen on skeletal surveys in up to 20%”.
3. 12.03.34 Patient with Waldenstroms macroglobulinaemia, which is most correct ? (–) (GC)
4. Bone lesions indistinguishable from multiple myeloma (false)
5. Bone lesions in <3% - T - unlike myeloma, lytic bone lesions are “uncommon” (eMed). Robbins says “does not produce lytic bone lesions”; Dahnert says “lytic lesions seen on skeletal surveys in up to 20%”.
6. Bone lesions involving cortex - F - marrow infiltration.
7. Solitary Bone lesion most likely similar sites as multiple myeloma but spares skull (false)
8. Subperiosteal deposits – endosteal spread (false)

Lymphoplasmacytic lymphoma is a low grade lymophoid malignancy composed of mature plasmacytoid lymphocytes.
Production of abnormal monocloncal IgM protein leads to Waldenstrom’s MG (hyperviscosity syndrome).
Sx: visual impairment (retinal vein distension, retinal h’ages), headaches/dizziness/deafness, bleeding, cryoglobulinaemia (Raynaud, cold urticaria).
BM infiltration in >90%, may be diffuse or variegated replacement. Lymphadenopathy, hepatosplenomegaly.
M-spike on serum/urine EP, no BJ proteinuria.
Added “which is most correct” to Qu.

Question 16

18. 12.03.93 What is the MOST CONTROVERSIAL (and rarest ?) subgroup in Hodkins lymphoma classification?
19. Nodular sclerosing
20. Lyphocyte predominant
21. Lymphocytic proliferative (not a subgroup)
22. Lyphocyte deplete
23. Mixed cellularity

Answer 16

4. Lyphocyte deplete (very rare)
5. 12.03.93 What is the MOST CONTROVERSIAL (and rarest ?) subgroup in Hodgkins lymphoma classification? Rob p431 (–)
6. Nodular sclerosing
7. Lyphocyte predominant
8. Lymphocytic proliferative (not a subgroup)
9. Lyphocyte deplete (very rare)
10. Mixed cellularity
    There are three major subtypes of HD:
    • Nodular sclerosis
    • Mixed cellularity
    • Lymphocyte predominance
    Additional variants, such as lymphocyte depletion HD, are rare and controversial entities

Question 17

19. 12.02.12 What is the staging of Hodgkin’s lymphoma which involve cervical, mediastinal and crural nodes?
20. Stage I
21. Stage II
22. Stage III
23. IB
24. IIS

Answer 17

2. Stage II

Question 18

20. 12.03.33 Patient with Hodgins disease Stage III ES which is NOT TRUE?
21. Nodes above diaphragm
22. Nodes below diaphragm
23. Involvement of extranodal sites
24. Involvement of spleen
25. Involvement of liver

Answer 18

5. Involvement of liver
6. 12.03.33 Patient with Hodgkins disease Stage III ES which is NOT TRUE? (–) (GC)
7. Nodes above diaphragm ] Both sides of the diaphragm
8. Nodes below diaphragm ] Both sides of the diaphragm
9. Involvement of extranodal sites - T - limited contiguous extralymphatic organ or site, as denoted by the E
10. Involvement of spleen - T - splenic involvement, as denoted by the S
11. Involvement of liver

Question 19

21. 12.03.29 Patient with polythaemia ruba vera which is NOT TRUE?
22. Transform to AML
23. Transform to CML
24. Develop/Associated with myelofibrosis
25. Associated with splenomegaly and cirrhosis
26. Risk of stroke
27. Associated with Budd-Chiari

Answer 19

2. Transform to CML - F
3. 12.03.29 Patient with polythaemia ruba vera which is NOT TRUE? Rob p439 (GC)
4. Transform to AML - T - blast crisis occurs spontaneously in 2% of pts treated with phlebotomy alone; in 15% who receive myelosuppresive treatment.
5. Transform to CML - F
6. Develop/associated with myelofibrosis - T - “spent phase” - 20% progress
7. Associated with splenomegaly and cirrhosis - T - hepatosplenomegaly (foci of extramedullary haematopoeisis), thrombosis (eg. Budd-Chiari syndrome, MI), infarcts (eg. CVA, spleen, kidney), haemorrhages, pruritis (histamine from basophils), gout.
8. Risk of stroke - T - see above.
9. Associated with Budd-Chiari - T - see above.

Question 20

22. 12.03.32 Patient with abnormal bleeding, normal platelets but decreased factor VIII + cofactor ristocetin – DIAGNOSIS is?
23. Haemophilia A (Factor VIII deficiency, Classic Haemophilia)
24. Haemophilia B (Factor IX defiency, Christmas disease)
25. Von Willebrands disease
26. Lupus anticogulation
27. DIC

Answer 20

3. Von Willebrands disease
4. 12.03.32 Patient with abnormal bleeding, normal platelets but decreased factor VIII + cofactor ristocetin – DIAGNOSIS is? Rob p449 (–)
5. Haemophilia A (Factor VIII deficiency, Classic Haemophilia)
6. Haemophilia B (Factor IX defiency, Christmas disease)
7. Von Willebrands disease
8. Lupus anticogulation
9. DIC
   • Patients with von Willebrand disease have prolonged bleeding time with normal platelet count. The plasma level of vWF, measured as the ristocetin cofactor activity, is reduced. Because vWF stabilizes factor VIII by binding to it, a deficiency of vWF gives rise to a secondary decrease in factor VIII levels

Question 21

23. 12.03.37 DEFINITION of Thymoma
24. Any benign thymic tumour
25. Benign tumour of epithelial cells
26. Any tumour of thymic epithelial cells
27. Any tumour of thymus
28. Benign tumours of epithelial and mesenchymal origin

Answer 21

3. Any tumour of thymic epithelial cells

Question 22

24. Sep03.07 AML
25. Philadelphia chromosome
26. Most common in children

Answer 22

both false

1. Most common in children (primarily affects adults 15-39 years, ALL is the one which usually affects children)
2. Philadelphia chromosome
   • primarily affects adults 15-39 years
   • accounts for only 20% of childhood leukaemias
   • Predisposing conditions
   o Alkylating agents
   o Myeloproliferative disorders
   o Aplastic anaemia
   o PNH
   • lymphadenopathy + hepatosplenomegaly due to infiltration by leukemic cells, less than ALL
   • meningeal infiltration - headaches, nausea, vomiting, papilloedema, CN palsies, seizures, coma
   • intracerebral / subarachnoid haemorrhages from thrombocytopenia + leukostasis, less than ALL
   • 60-80% show remission with chemotherapy but relapse within 12-18 months
   • 15 – 30 % 5 yr survival
   • Prognosis influenced by chromosomal abnormalities:
   o t(8;21) or inversion of 16 = good
   o t(9;22) Ph or 11q23 = bad
   o Philidelphia chromosome usually in CML but does occur in AML and is associated with a bad prognosis

Question 23

25. Sep03.43 Megaloblastic anaemia is not caused by :
26. Pernicious anaemia
27. Crohn’s disease
28. Coeliac disease
29. Pancreatic calcification

Answer 23

4. Pancreatic calcification (malabsorption ?)

**LJS - pancreatic insufficiency does cause B12 def. Coeliac most incorrect? Duodenum and jejunum mostly affected

25. Sep03.43 Megaloblastic anaemia is not caused by: (–)
26. Pernicious anaemia
27. Crohn’s disease
28. Coeliac disease
29. Pancreatic calcification (malabsorption ?)
    The two most common causes of megaloblastic anemia are vitamin B-12 deficiency (cobalamin) and folinic acid deficiency.
    In the cell:
    • cobalamin acts as a coenzyme in 2 reactions: the synthesis of methionine from homocysteine and the conversion of methylmalonyl CoA to succinyl CoA.
    • folate binds to and acts as a coenzyme with enzymes responsible for single carbon metabolism.

Vitamin B-12 is commonly ingested with meat or fish. It binds to salivary haptocorrins, which are digested in the stomach, allowing the cobalamin to bind to intrinsic factor (IF). IF is produced by the parietal cells of the stomach. The IF-cobalamin complex makes its way to the terminal ileum, where it binds to receptors on the enterocyte. It is transported across the cell and enters the circulation bound to a transport molecule, TC II. The cobalamin-TC II complex is absorbed into cells by endocytosis.
Vitamin B-12 deficiency can be caused by decreased ingestion (eg, poor dietary intake), impaired absorption (eg, failure to release B-12 from protein, intrinsic factor [IF] deficiency, chronic pancreatic disease, competitive parasites, intrinsic intestinal disease), or impaired utilization (eg, congenital enzyme deficiencies, lack of transcobalamin II, administration of nitrous oxide).
Inadequate B-12 dietary intake is extremely rare in children. Pernicious anemia, the most common cause of B-12 deficiency in adults, is rare in childhood. Deficiency of vitamin B-12 activity is usually due to intestinal malabsorption or a congenital deficiency of one of the vitamin B-12 carrier proteins. In recent years, vitamin B-12 deficiency has been described in patients with HIV infection, with or without AIDS.
In addition to the hematologic manifestations of vitamin B-12 deficiency, abnormalities of the gastrointestinal tract and nervous system are also present. The underlying cause of megaloblastic anemia must be determined in each case. Failure to recognize B-12 deficiency, even in the presence of concomitant folate deficiency, may result in permanent neurologic damage. Treatment with folate alone in these cases may aggravate this situation.

Folate is ingested in the diet in many different types of food. It enters the enterocyte and is transported into the portal circulation by a carrier molecule. It circulates in the plasma as, mostly, 5 methyl tetrahydrofolate (THF). It enters the cell via a carrier (methotrexate competes with this carrier).
Folate deficiency can be caused by decreased ingestion (eg, poor dietary intake, alcoholism, infancy), impaired absorption (eg, intestinal short circuits, tropical sprue, congenital malabsorption, certain drugs such as sulfasalazine), impaired utilization (eg, use of folic acid antagonists such as methotrexate), increased requirements (eg, pregnancy, infancy, hyperthyroidism, chronic hemolytic disease, cancer), or increased loss (eg, hemodialysis).
Folic acid is available in a wide variety of food groups. Approximately a third of dietary folate is estimated to come from cereals and grains, another third from fruits and vegetables, and another third from meats and fish. Folic acid deficiency is commonly observed in children who are fed a severely restricted diet. This usually occurs with a diet restricted to goat’s milk, which is deficient in folic acid. It may also be observed in children with celiac sprue and other malabsorption disorders that affect the proximal small intestine.
Deficiency of metabolically active folate metabolites is frequently observed in patients who receive antifolate drugs, such as sulfa antibiotics and methotrexate. A relative deficiency of metabolically active folate metabolites may also be observed in patients who are experiencing increased red cell destruction.

Question 24

26. Sep03.58 Sickle cell anaemia. An unusual feature would be
27. splenomegaly
28. splenic atrophy/ absent
29. fungal dactylitis
30. aplastic crises
31. bone infarction

Answer 24

3. fungal dactylitis - F
4. Sep03.58 Sickle cell anaemia. An unusual feature would be: (GC)
5. splenomegaly - T - in children, during the early phase of the disease, the spleen is commonly enlarged up to 500g
6. splenic atrophy/ absent - T - marked congestion of the red pulp, due mainly to the trapping of sickled red cells in the splenic cords and sinuses. Erythrostasis in the spleen leads to thrombosis and infarction, or at least to marked tissue hypoxia. Continued scarring causes progressive shrinkage of the spleen so that by adolescence or early adulthood, only a small nubbin of fibrous tissue may be left; ie. autosplenectomy
7. fungal dactylitis - F
8. aplastic crises -T - aplastic crisis represents a temporary cessation of bone marrow activity, usually triggered by parvovirus infection of erythroid progenitor cells. Reticulocytes disappear from the peripheral blood, and there is sudden and rapid worsening of anemia.
9. bone infarction -T - in children, painful bone crises are extremely common and often difficult to distinguish from acute osteomyelitis. They frequently manifest as the hand-foot syndrome because of dactylitis of the bones of the hands or feet or both.

Question 25

27. 33.APRIL02 A 34-week obstetric ultrasound is requested to assess fetal growth. The clinical information states says “Mother “O” /father “AB”, Haemolytic disease on the newborn (HDNP)??”They have had no counselling and are worried about damage to this, their second baby. What is the best summary of their situation?
28. There is no risk of HDN from ABO incompatibility
29. There is no risk of HDN with their given blood groups
30. The risk depends how the last delivery was managed but may approach 100%.
31. Only 1 in 200 cases at risk of ABO related disease require treatment
32. Only 1 in 10 cases at risk of ABO related disease develop HDN, only 1 in 200 require treatment

Answer 25

5. Only 1 in 10 cases at risk of ABO related disease develop HDN, only 1 in 200 require treatment

• Erythroblastosis fetalis is defined as a hemolytic disease in the newborn caused by blood-group incompatibility between mother and child
• When the fetus inherits red cell antigenic determinants from the father that are foreign to the mother, a maternal immune reaction may occur, leading to hemolytic disease in the infant. Basic to such a phenomenon are leakage of fetal red cells into the maternal circulation and, in turn, transplacental passage of maternal antibodies into the fetus. Any of the numerous red cell antigenic systems may theoretically be involved, but the major antigens known to induce clinically significant immunologic disease are the ABO and certain of the Rh antigens.
• Concurrent ABO incompatibility protects the mother against Rh immunization because the fetal red cells are promptly coated by isohemagglutinins and removed from the maternal circulation.
• The antibody response depends on the dose of immunizing antigen; hence, hemolytic disease develops only when the mother has experienced a significant transplacental bleed (more than 1 ml of Rh-positive red cells).
• The istotype of the antibody is important because immunoglobulin G (IgG) (but not immunoglobulin M [IgM]) antibodies can cross the placenta. The initial exposure to Rh antigen evokes the formation of IgM antibodies, so Rh disease is uncommon with the first pregnancy. Subsequent exposure during a second or third pregnancy generally leads to a brisk IgG antibody response.
• The pathogenesis of fetal hemolysis caused by maternal/fetal ABO incompatibility is slightly different than that caused by differences in the Rh antigens.
o ABO incompatibility occurs in approximately 20 to 25% of pregnancies, but laboratory evidence of hemolytic disease occurs only in 1 in 10 of such infants, and the hemolytic disease is severe enough to require treatment in only 1 in 200 cases.
o Several factors account for this.
o First, most anti-A and anti-B antibodies are of the IgM type and hence do not cross placenta.
o Second, neonatal red cells express blood group antigens A and B poorly.
o Third, many cells other than red cells express A and B antigens and thus sop up some of the transferred antibody.
o ABO hemolytic disease occurs almost exclusively in infants born to group O mothers because some have IgG anti-A and anti-B antibodies without obvious sensitization.
o There is no effective protection against ABO reactions.

Question 26

28. 58.APRIL02 A 24-year-old woman with lymphoma is seriously unwell after 2 weeks of , headache. Over the last 24 hours she has developed blackened areas of skin over her face, and a right proptosis. CT shows non-specific oedema of the face and orbit, diffuse sinus opacity, and absence of flow in the left cavernous sinus. Cultures of nasal swabs show non-septate irregular hyphae with right angle branches. The most likely organism is
29. Apergillosus
30. Mucormycosis
31. Cryptococcus
32. Candida
33. Listeria

Answer 26

b. Mucormycosis – T – Phycomycetes (Mucor, Absidia, Rhizopus, Cunninghamella) form nonseptate, irregularly wide (6-50um) fungal hyphae with frequent right-angle branching.

28. 58.APRIL02 A 24-year-old woman with lymphoma is seriously unwell after 2 weeks of , headache. Over the last 24 hours she has developed blackened areas of skin over her face, and a right proptosis. CT shows non-specific oedema of the face and orbit, diffuse sinus opacity, and absence of flow in the left cavernous sinus. Cultures of nasal swabs show non-septate irregular hyphae with right angle branches. The most likely organism is (–) (TW)
    a. Apergillosus – F – aspergillus form fruiting bodies (esp in cavities) and septate filaments, 5-10um thick, branching at acute angles (40 degrees)
    b. Mucormycosis – T – Phycomycetes (Mucor, Absidia, Rhizopus, Cunninghamella) form nonseptate, irregularly wide (6-50um) fungal hyphae with frequent right-angle branching.
    c. Cryptococcus – F – encapsulated yeast. Produced buds that characteristically are narrow-based and the organism is surrounded by a polysaccharide capsule.
    d. Candida – F – polymorphic yeast (ie yeast cells, hyphae and pseudohyphae are produced). Pseudohyphae may be formed from budding yeast cells that remain attached to each other.
    e. Listeria – F – gram positive, motile facultative intracellular bacterium.Gram positive rods.
    • Mucormycosis is an opportunistic infection of neutropenic persons and ketoacidotic diabetics caused by “bread mold fungi,” including Mucor, Absidia, Rhizopus, and Cunninghamella, which are collectively referred to as the Phycomycetes.
    • These fungi, which are widely distributed in nature and cause no harm to immunocompetent individuals, infect immunosuppressed patients somewhat less frequently than do Candida or Aspergillus.
    • The three primary sites of Mucor invasion are the nasal sinuses, lungs, and gastrointestinal tract, depending on whether the spores (widespread in dust and air) are inhaled or ingested. In diabetics, the fungus may spread from nasal sinuses to the orbit, and brain, giving rise to rhinocerebral mucormycosis.
    • The Phycomycetes cause local tissue necrosis, invade arterial walls, and penetrate the periorbital tissues and cranial vault.
    • Meningoencephalitis follows, sometimes complicated by cerebral infarctions when fungi invade arteries and induce thrombosis. Phycomycetes form nonseptate, irregularly wide (6 to 50 mum) fungal hyphae with frequent right-angle branching, which are readily demonstrated in the necrotic tissues by hematoxylin and eosin or special fungal stains
    • Lung involvement with Mucor may be secondary to rhinocerebral disease, or it may be primary in patients with hematologic neoplasms. The lung lesions combine areas of hemorrhagic pneumonia with vascular thrombi and distal infarctions.

Question 27

29. Sep03.77 Procedure planned, what is the least likely risk factor for DIC
30. Prostatic cancer
31. Giant haemangioma
32. Sarcoidosis
33. Placental abruption

Answer 27

3. Sarcoidosis

MAJOR DISORDERS ASSOCIATED WITH DISSEMINATED INTRAVASCULAR COAGULATION
Obstetric Complications
Abruptio placentae
Retained dead fetus
Septic abortion
Amniotic fluid embolism
Toxemia

Infections
Gram-negative sepsis
Meningococcemia
Rocky Mountain spotted fever
Histoplasmosis
Aspergillosis
Malaria

Neoplasms
Carcinomas of pancreas, prostate, lung, and stomach
Acute promyelocytic leukemia
Massive Tissue Injury
Traumatic
Burns
Extensive surgery

Miscellaneous
Acute intravascular hemolysis, snakebite, giant hemangioma, shock, heat stroke, vasculitis, aortic aneurysm, liver disease

Question 28

30. PATH2004 Follicular lymphoma - incorrect
31. Common 15-40 year olds
32. Nodular growth, follicular B cells
33. T(14;18) translocation
34. Transformation occurs to diffuse large B-cell lymphoma in 30 – 50%, rarely to aggressive form of lymphoblastic lymphoma or leukaemia
35. Is characterised by cells in stroma that can change to diffuse appearance
36. low grade on old working classification
37. Is indolent but incurable
38. Good survival
39. Poor chemo response
40. commonly arises in gut

Answer 28

30. PATH2004 Follicular lymphoma – incorrect (TW) (–)
31. Common 15-40 year olds – F - frequently presents in middle-aged individuals and the elderly with a median age at Dx of 60.
32. Nodular growth, follicular B cells – T - lymphoma of follicle center B-cells (centrocytes and centroblasts), which has at least a partially follicular pattern.
33. T(14;18) translocation – T – Approx 85% of pts with FL have t(14;18) which results in the overexpression of BCL-2, an oncogene that blocks programmed cell death.
34. Transformation occurs to diffuse large B-cell lymphoma in 30 – 50%, rarely to aggressive form of lymphoblastic lymphoma or leukaemia – T
35. Is characterised by cells in stroma that can change to diffuse appearance – T - 30-50% transformation occurs to diffuse large B-cell lymphoma
36. low grade on old working classification – T – considered an indolent NHL, but incurable.
37. Is indolent but incurable - T
38. Good survival - ?T?F – depends on Early or Late stage presentation, plus treatment. Proportion also have transformation to higher grade lymphoma per year.
39. Poor chemo response – T
40. commonly arises in gut – F – involvement of organs other than the lymphatic organs or bone marrow is uncommon.
    • Follicular lymphoma is the most common form of NHL in the United States, comprising about 45% of adult lymphomas. It usually presents in middle age and afflicts men and women equally. It is less common in Europe and rare in Asian populations. The neoplastic cells closely resemble normal germinal center B cells.
    o The overall median survival is 7 to 9 years and is not improved by aggressive therapy hence, the usual clinical approach is to palliate patients with low-dose chemotherapy or radiation when they become symptomatic
    • Although follicular lymphoma is incurable, it often follows an indolent waxing and waning course.

Question 29

31. PATH2004 Regarding lymphoma
32. Lymphocyte depleted is most common
33. RS cell is derived from B cell
34. HD and NHD often coexist
35. FNA cannot Dx HD as it is the architecture of lymph node that makes the Dx

Answer 29

2. RS cell is derived from the B cell - T – probably best answer seeing they emphasise this point at the path course. Neoplastic RS cells are now considered to be B-cells, typically CD30 / CD15 positive, and CD 20 / EMA / CD45 negative. They are not classified as B-cell lymphomas as they don’t act like them. Molecular studies have established a B-cell origin for RS cells in the vast majority of cases of HL.
3. PATH2004 Regarding lymphoma (TW & GC)
4. Lymphocyte deplete is the most common form - F - rare entity. Nodular sclerosis type is most common.
5. RS cell is derived from the B cell - T – probably best answer seeing they emphasise this point at the path course. Neoplastic RS cells are now considered to be B-cells, typically CD30 / CD15 positive, and CD 20 / EMA / CD45 negative. They are not classified as B-cell lymphomas as they don’t act like them. Molecular studies have established a B-cell origin for RS cells in the vast majority of cases of HL.
6. HD and NHD often coexist - F - can occur, but not often. “Composite lymphoma” - term used to denote 2 distinct types of lymphoma occurring within a single organ or tissue. Synchronous occurrence of 2 types of NHLs is more common than an NHL and HL (But occurs).
7. FNA cannot diagnose HD as it is the architecture of the lymph node that makes the diagnosis – F(?T) - can Dx with FNA (RS cells). Lymph node excision Bx is used for determining type which is based on morphologic and immunophenotypic features.
   Path course stressed that RS cell is derived from the B-cell at the path course.
   **LJS - from Mrs Mike Hurrel (pathologist) - need cells for lymphoma diagnosis - for flow cytometry, not tissue. Robbins says this also. So can make diagnosis with FNA

Question 30

1. Which is not a feature of Follicular lymphoma?
2. Prolonged survival 7-9 yrs .
3. Incurable.
4. Occurs between ages of 15-40 yrs .
5. 30% of patients die due to transformation into high grade disease.

Answer 30

3. Occurs between ages of 15-40 yrs - F – middle-aged individuals and the elderly, with a median age at Dx of 60. Robbins: occurs in older persons, rare <20.
4. Which is not a feature of Follicular lymphoma? (TW & GC)
5. Prolonged survival 7-9 yrs - F – varies. If Dx in Early-Stage disease can have prolonged survivial >20y, if (as are the majority) Dx in Advanced-Stage less, but depending on type of Rx. Path notes say “Follicular lymphoma is not curable, and 10yr survival is poor.
6. Incurable – T – the indolent NHLs are usually not curable with conventional treatment (UTD). Path notes: Incurable disease.
7. Occurs between ages of 15-40 yrs - F – middle-aged individuals and the elderly, with a median age at Dx of 60. Robbins: occurs in older persons, rare <20.
8. 30% of patients die due to transformation into high grade disease – T – variable rate of histological transformation (10-70%) depending on definition of histological transformation. Generally thought to be 1.4 to 4.4% per year (UTD). Robbins: in about 40% FL progresses to a DLBCL, with or without treatment
   Follicular lymphoma is the most common of the indolent NHLs. It is defined as a lymphoma of the follicle centre B-cells (centrocytes and centroblasts), which has at least a partially follicular pattern.
   Comprises approx 20% of all NHLs, and up to 70% of “low grade” lymphomas (UTD).
   Path notes: Paradox, in that a very aggressive disease, eg Anaplastic large cell lymphoma, if response to the treatment administered, actually has a better long-term prognosis than a low grade disease: Follicular lymphoma is not responsive to treatment and not curable.

Question 31

2. Hodgkins VS Non hodgkins lymphoma?
3. isolated regional nodes.
4. Involves waldeyers ring.
5. Mesenteric node involvement.

Answer 31

2. Hodgkins VS Non hodgkins lymphoma? (TW)
3. isolated regional nodes – HL arises in a single node or chain of nodes and spreads characteristically to the anatomically contiguous nodes.
4. Involves waldeyers ring – nodal and extranodal involvement of the head and neck, including Waldeyer’s ring (tonsils, base of tongue, nasopharynx) is more frequently observed in patients with NHL than in HL.
5. Mesenteric node involvement – involvement of retroperitoneal, mesenteric, and pelvic nodes is common in most histologic subtypes of NHL. In HL, exclusive infradiaphragmatic lymphadenopathy is infrequent, occurring in less than 10% (UTD).

Question 32

3. 60 yr old female with follicular lymphoma which is false?
4. Involves liver and spleen.
5. Follicles and nodules on histology.
6. Disease waxes and wanes with treatment.
7. Atypical sex and age.

Answer 32

*LW:
Favoured answer is involves liver and spleen which are incorrect.
Robbins states:
- Predominantes in older person, equal gender. (so typical for age and sex.
- Histology of nodular appearance
- Visceral site involvement is uncommon.
LJS - came here to say the same thing

Previous answers:
4. Atypical sex and age – F – frequently presents in middle-aged individuals and the elderly with a median
age at Dx of 60. Slight female predominance (1.7:1 F:M). Rarely, follicular lymphoma arises in children or adolescents.

3. 60 yr old female with follicular lymphoma which is false? (TW)
4. Involves liver and spleen – T – involves spleen in 50% and liver in 50% of cases.
5. Follicles and nodules on histology – T – lymphoma of follicle center B-cells (centrocytes and centroblasts), which has at least a partially follicular pattern.
6. Disease waxes and wanes with treatment - T
7. Atypical sex and age – F – frequently presents in middle-aged individuals and the elderly with a median age at Dx of 60. Slight female predominance (1.7:1 F:M). Rarely, follicular lymphoma arises in children or adolescents.

Question 33

4. Which is true regarding Hodgkins lymphoma?
5. Waldeyers rin involvement common.
6. FNA can’t diagnose condition need to look at node architecture.
7. Reed Sternberg cells derived from abnormal B cells.

Answer 33

3. Reed Sternberg cells derived from abnormal B cells – T - neoplastic R/S cells are now considered to be B-cells, typically CD30 / CD15 positive, and CD 20 / EMA / CD45 negative. They are not classified as b-cell lymphomas as they don’t act like them. Molecular studies have established a B-cell origin for RS cells in the vast majority of cases of HL.
4. Which is true regarding Hodgkins lymphoma? (TW)
5. Waldeyers ring involvement common – F - nodal and extranodal involvement of the head and neck, including Waldeyer’s ring (tonsils, base of tongue, nasopharynx) is more frequently observed in patients with NHL than in HL.
6. FNA can’t diagnose condition need to look at node architecture – ?T – probably not best answer and is repeated question, however can Dx with FNA (RS cells). Lymph node excision Bx is used for determining type which is based on morphologic and immunophenotypic features.
7. Reed Sternberg cells derived from abnormal B cells – T - neoplastic R/S cells are now considered to be B-cells, typically CD30 / CD15 positive, and CD 20 / EMA / CD45 negative. They are not classified as b-cell lymphomas as they don’t act like them. Molecular studies have established a B-cell origin for RS cells in the vast majority of cases of HL.
   This question is repeated later and I have altered option 3. to be false. Both 2. and 3. are T. The initial remembered option is as this question is presented. They seemed to stress that RS cell is derived from the B-cell at the path course - so this would probably be the most likely answer.

Question 34

1. Hospital pathology computer shows that the serum electrophoretic analysis and urinary Bence Jones protein assessment are normal. Which of the following statements is most correct:
2. The findings exclude multiple myeloma
3. 1 in 100 cases of multiple myeloma would fit this pattern
4. 20% of case of multiple myeloma would fit this pattern
5. Bence Jones protein assessment should have done on serum
6. In approximately 70% of patients the electrophoretic pattern alone is normal

Answer 34

2. 1 in 100 cases of multiple myeloma would fit this pattern – T – probably most true. Nonsecretory MM in ~3%.
3. Hospital pathology computer shows that the serum electrophoretic analysis and urinary Bence Jones protein assessment are normal. Which of the following statements is most correct: (TW)
4. The findings exclude multiple myeloma – F
5. 1 in 100 cases of multiple myeloma would fit this pattern – T – probably most true. Nonsecretory MM in ~3%.
6. 20% of case of multiple myeloma would fit this pattern – F – nonsecretory MM 3%.
7. Bence Jones protein assessment should have done on serum – F – in 16% of patients with MM, only a Bence Jones protein (light chain) was produced, often too low to be detected by routine immunofixation techniques. These tend to be concentrated in urine.
8. In approximately 70% of patients the electrophoretic pattern alone is normal – F – the most important diagnostic finding in MM is the demonstration of a monoclonal protein, which is present in the serum and/or urine in 97% of patients.
   Multiple myeloma is characterized by the neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin.
   Approximately 3% of patients with MM have no M-protein in the serum or urine on immunofixation at the time of Dx and are considered to have nonsecretory myeloma. The condition remains nonsecretory in most patients (76%).

Question 35

1. Small round blue cell tumours. What is the least likely site in a child?
2. Eye
3. Kidney
4. Adrenal
5. Testis
6. Brain

Answer 35

4. Testis - F – but can sometimes get desmoplastic small round cell tumor in a paratesticular location.
5. Small round blue cell tumours. What is the least likely site in a child? (TW)
6. Eye - T – sheets, trabeculae and nests of small blue cells with scant cytoplasm.
7. Kidney - T - Wilms
8. Adrenal - T - neuroblastoma
9. Testis - F – but can sometimes get desmoplastic small round cell tumor in a paratesticular location.
10. Brain - T - medulloblastoma
    Owing to their primitive histologic appearance, many childhood tumors have been collectively referred to as small round blue cell tumors. These are characterised by sheets of cells with small, round nuclei. The differential diagnosis of such tumors includes neuroblastoma, lymphoma, rhabdomyosarcoma, Ewing sarcoma, and occasionally Wilms’ tumor.

Question 36

2. Which system is currently used for lymphoma classification?
3. WHO
4. Real
5. Rappoport
6. Kiel
7. Working

Answer 36

1. WHO – T – in 1994, the International Lymphoma Study Group introduced an updated classification, incorporating new immunologic and molecular data, as part of the Revised European-American Lymphoma (REAL) classification of lymphomas. These concepts have been incorporated into the new World Health Organization (WHO) classification of hematologic malignancies prepared as a joint effort of the Society for Hematopathology and the European Association of Hematopathologists.

Question 37

3. Which is not a type of Hodgkins lymphoma?
4. Lymphocyte rich
5. Lymphocyte predominant
6. Lymphocyte depleted
7. Mixed cellularity
8. Anaplastic

Answer 37

5. Anaplastic – F - anaplastic large cell lymphoma (usually made up of T-lymphocytes)
6. Which is not a type of Hodgkins lymphoma? (GC, TW)
7. Lymphocyte rich – T - rare
8. Lymphocyte predominant - T - young adult males. Hx: L+H (popcorn) variant of RS cell, panB-cell markers +. Excellent prognosis; 3-5% transform into DLBCL.
9. Lymphocyte depleted - T - rare and somewhat controversial. Most cases prove to be large cell lymphoma. Older male patients with disseminated disease, HIV patients, developing countries.
10. Mixed cellularity - T - older men. Hx: lots of RS cells and heterogeneous infiltrate. Usually disseminated disease + B symptoms.
11. Anaplastic – F - anaplastic large cell lymphoma (usually made up of T-lymphocytes)

Question 38

In regard to the clinical presentation of multiple myeloma, which is the LEAST correct?

a. Over 90% of patients have increased levels of immunoglobulins in the blood
b. Progression to classic MM is common in solitary osseous plasmacytoma
c. Renal insufficiency is a common contributor to death
d. Impaired cellular immunity predisposes to recurrent viral infections
e. Untreated prognosis is poor

Answer 38

**LJS - I think d. least correct.
Impaired humoral immunity due to lack of antigen diversity (monoclonal plasma cell population producing only one Ab type).

d. impaired cellular immunity predisposes to recurrent viral infections - F - “Decreased production of normal Igs sets the stage for recurrent bacterial infections. Cellular immunity is relatively unaffected.” Robbins pg 600. *RY (previous answer a)
* LW: agrees with the ladies!

In regard to the clinical presentation of multiple myeloma, which is the LEAST correct

a. Over 90% of patients have increased levels of immunoglobulins in the blood F more like 80%
b. Progression to classic MM is common in solitary osseous plasmacytoma
c. Renal insufficiency is a common contributor to death - T “ Of great significance is renal insufficiency, which trails only infections as a cause of death… …occurs in 50% of patients.” (Robbins)
d. Impaired cellular immunity predisposes to recurrent viral infections
e. Untreated prognosis is poor

• Protein electrophoresis:
  o Serum protein electrophoresis reveals:
   Paraprotein – monoclonal Ig producing high ‘M’ spike (IgG 55%, IgA 25%), &/or ↓ concentrations of normal immunoglobulins (immune paresis)
    20% only produce light chains  no plasma M band will be found
  o Bence-Jones proteins (light chains) in the urine – the only finding in 20%
  o Statistics on paraproteins in MM:
   Presence in blood, urine, or both = 99%
   Both blood and urine = 60 to 70%
   Urine alone 20% (light chains only)
   Non secretory 1% (‘nonsecretors’ – have no identifiable M protein, urine or plasma  the absence of paraproteinemia and paraproteinuria does not exclude myeloma). PATH MCQ