IM1 LE3 Flashcards
You cannot give glucocorticoids to the following patients, except: A. Leukemia B. Castleman's disease C. Lymphoma D. Infectious mononucleosis
D. Can cause pharyngeal obstruction by enlarged lymphoid tissue in the Waldeyer’s ring; can be life-threathening
One can say that lymphadenopathy requires biopsy, when there is/are: (risk factors)
> 40 yo, supraclavicular location, size is >2.25cm2, hard texture, nontender/nonpainful
True or False: Most lymphadenopathy requires biopsy.
False. Most of them are benign. Most studies shows that CBC is more useful.
CBC is useful for the diagnosis of the following, except: A. Acute Leukemia B. Chronic Leukemia C. SLE D. EBV/CMV mononucleosis
C. Should be ANA
Thoracic adenopathy can be detected by:
Chest radiography or during workup for superficial adenopathy
The following are not function of spleen, except:
A. Synthesis of antigen in the white pulp
B. Synthesis of antibodies in the red pulp
C. Maintenance of quality control of erythrocytes in the white pulp
D. Maintenance of quality control of erythrocytes in the red pulp
D. By removal of senescent and defective RBC
True or False: Spleen sequester and store RBC.
False. Spleen does not sequester and store RBC. Animals do. In humans, spleen contains 1/3 of total body PLT and marginated neutrophils. These sequestered cells are available to respond to bleeding or infection.
Massive splenomegaly can cause:
Satiety
True or False: The rupture of spleen is painful.
False. Its rupture is usually painless.
Palpation of the spleen is accomplished by:
Middleton maneuver (bimanual palpation, ballotment, palpation from above)
Percussion of spleen can be done by the following, except: A. Nixon's B. Middleton's C. Castell's D. Barkun's
B. Middleton’s - Palpation
Procedure of choice for routine assessment of spleen
Ultrasound
Erythrocyte count is increased in: A. Polycythemia vera B. SLE C. Thalassemia major D. Felty's syndrome
A. Correct: All blood cells are increased
B. Normal, decreased
C. Normal, decreased
D. Granulocyte: Normal, decreased
When can you say there is a splenomegaly?
Spleen:
- Extends >8 cm below the left costal margin
- Weighs >1000 g
Only contraindication to splenectomy:
Marrow failure, in which enlarged spleen is the only source of hematopoietic tissue
2 weeks pre-splenectomy vaccination includes:
Pneumococcal conjugate vaccine - more effective
N. meningitidis vaccine - may also be given
HiB vaccine - post-splenectomy
Coexistence of this disease with splenomegaly can cause hemoglobinopathies, such as thalassemia or hemoglobin C:
Sickle cell disease
The following are causes of JAK2 mutation, except: A. Chronic myelogenous leukemia B. Polycythemia vera C. Primary myelofibrosis D. Essential thrombocytosis
A. Balanced translocation between chromosomes 9 and 22; t(9;22)
Most common myeloproliferative disorder:
Polycythemia vera: Familial, women predominate in sporadic cases
Etiology of polycythemia vera:
Unknown
Most common cytogenetic abnormality in polycythemia vera:
JAK2 located in chromosome 9 and loss of heterozygosity on chromosome 9p (due to mitotic recombination) –> Leads to homozygosity
Initial presenting sign of polycythemia vera: A. Hepatomegaly B. Splenomegaly C. Aquagenic pruritus D. High hemoglobin or hematocrit
B. Correct
A. No association
C. Assoc. with erythrocytosis
D. First recognized but splenomegaly is the true initial
Erythromelalgia is a symptom complex composed of, except: A. Myalgia B. Erythema C. Burning sensation D. Pain
A.
Notes: Erythromelalgia is a complication of thrombocytosis in polycythemia vera due to platelet stickiness
Large turnover of hematopoietic cells in polycythemia vera can lead to: A. Hypokalemia B. Hyperuricemia C. Metabolic alkalosis D. Hypophosphatemia
B. Correct. It can also lead to uric acid stones, hypermetabolism.
True about Gaisbock’s Syndrome:
A. Elevated red cell mass with normal plasma volume
B. Elevated red cell mass with high plasma volume
C. Decreased red cell mass with high plasma volume
D. Decreased red cell mass with normal plasma volume
B.
Notes: Seen in polycythemia vera; Red cell mass and plasma volume determinations are mandatory to establish the absolute erythrocytosis (to distinguish from relative erythrocytosis due to a reduction in plasma volume)
True or False: Arterial oxygen concentration is normal in polycythemia vera.
True
Notes: Hypoxic erythrocytosis has a low arterial O2 conc.
The following can cause microcytic erythrocytosis, except: A. a-thalassemia B. B-thalassemia C. Hypoxic erythrocytosis D. Polycythemia vera
A. Not associated
System abnormality that can mask the presence of polycythemia vera: A. Neurologic B. GU C. GI D. Musculoskeletal
C. GI: Peptic ulcer disease, related to bleeding, masks PV
True or False: BM aspiration and biopsy is needed to diagnose polycythemia vera.
False. Does not provide diagnostic info since it may be normal or indistinguishable from essential thrombocytosis and primary myelofibrosis
Causes of relative erythrocytosis, except: A. Carbon monoxide poisoning B. Dehydration C. Tobacco smoking D. Ethanol abuse
A. One of the causes of absolute erythrocytosis. Others include, polycythemia vera, sleep apnea syndrome, etc.
Check Table 108-2 of Chapter 108
Pruritus in polycythemia vera may be related to: A. Elevated erythrocytes B. Elevated platelets C. Elevated eosinophils D. Elevated basophils
D. Activation by JAK2 V617F
Most common complication of polycythemia vera (PV): A. Migraine B. Thrombosis C. Pruritus D. Ulcer
A. CNS symptom of PV
B. Correct; Secondary to erythrocytosis; It is important to maintain Hgb and Hct level
C. One of the symptoms due to basophil
D. GI symptoms that may mask PV
True or False: Salicylates can be used to treat thrombosis in polycythemia vera (PV)
False. It is harmful and not proven as a remedy
Notes: Anticoagulants are indicated
Allopurinol can be used to treat/reduce the following, except: A. Pruritus B. Splenomegaly C. Leukocytosis D. Thrombosis
D. Anticoagulants
A, B, C. Indicated but not always
Notes: Asymptomatic hyperuricemia in PV usually requires no therapy, but Allopurinol can be given to avoid further elevation
True or False: Splenomegaly requires no treatment even if symptomatic.
False. Splenomegaly can be treated with IFN-a, but can show side effects if used chronically.
Primary myelofibrosis is characterized by, except: A. Marrow fibrosis B. Medullary hematopoiesis C. Extramedullary hematopoiesis D. Splenomegaly
B.
Least common myeloproliferative disease: A. Polycythemia vera B. Primary myelofibrosis C. Essential thrombocytosis D. Chronic eosinophilic leukemia
B.
JP, a 63-year old male, smoker, drinks alcohol twice a day 5 days a week, and seems to be hypertensive upon PE. He was diagnosed to have agnogenic myeloid metaplasia. Which of the following predisposes him to this condition? A. Smoking B. Alcohol C. Hypertension D. Gender and age
D. Agnogenic myeloid metaplasia can occur at any age, but primarily afflicts men in their sixth decade or later.
Overproduction causes marrow angiogenesis in primary myelofibrosis?
A. Vascular endothelial growth factor
B. Osteoprotegerin
C. Transforming growth factor B
D. Tissue inhibitors of metalloproteinases
A. Correct
B. Overproduction associated with osteosclerosis; an osteoclast inhibitor
C and D. Overproduction associated with fibrosis
A 30 years old male came to your clinic with symptoms of weight loss, fatigue and night sweats. Upon examination, you figured out that he has splenomegaly. You knew that this presentations are unique to what myeloproliferative disorder? A. Chronic neutrophilic leukemia B. Polycythemia vera C. Tuberculosis D. Myelofibrosis with myeloid metaplasia
D. Also known as Primary Myelofibrosis
Notes: In contrast to its companion myeloproliferative disorders (MPDs), night sweats, fatigue and weight loss is common in PMF.
Teardrop-shaped red blood cells are seen in:
Primary myelofibrosis (PMF)
Notes: Addition to that, you can also see nucleated red cells, myelocytes, promyelocytes and myoblasts in PMF’s peripheral blood smear, indicative of extramedullary hematopoiesis.
What fibrosis of the bone marrow is secondary to a tumor/granulomatous process?
Myelophthisis
Notes: Myelofibrosis - fibrosis due to primary hematologic process
True or False: Primary myelofibrosis is associated with anemia.
True.
Notes: Anemia, usually mild initially.
Additional notes: Leukocytes and platelets are normal or increased, but may be depressed
Enzymes elevated in primary myelofibrosis: A. Lactate dehydrogenase B. Alkaline phosphatase C. Both D. Neither
C. Both
True or False: Primary myelofibrosis can be diagnosed by bone marrow aspiration.
False.
Notes: Marrow is not usually aspirable due to increased marrow reticulin (fibrosis), but MARROW BIOPSY will reveal hypercellular marrow with increased number of megakaryocytes in clusters and with large nuclei.
True or False: Primary myelofibrosis is associated with autoimmune abnormalities.
True.
Notes: Associated with immune complexes, ANA, rheumatoid factor, positive in Coombs’ test
This cluster of differentiation is increased in PMF, unlike any other MPDs:
CD 34+ cells
Notes: >15,000/uL - associated to myeloid metaplasia
Mutation with what gene is associated with anemia: A. JAK2 B. MPL C. GGCC D. JAK3
B.
What hematologic abnormality can exacerbate primary myelofibrosis? A. IDA B. Pernicious Anemia C. Folic acid deficiency D. Nonmegaloblastic anemia
C.
Therapy for primary myelofibrosis (PMF):
No specific therapy, but pyridoxine therapy has been effective in rare instances.
Anemia in PMF may be due to: A. GI blood loss B. Ineffective erythropoiesis C. Neither D. Both
D.
Proved useful in controlling organomegaly in PMF: A. Allopurinol B. Hydroxyurea C. Danazol D. Glucocorticoids
B.