Myeloproliferative Dx Flashcards Preview

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Flashcards in Myeloproliferative Dx Deck (65)
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0
Q

In myeloproliferative disorders each disorder is caused by what?

A

clonal expansion that arises from a pluripotent stem cell

1
Q

What are the 4 conditions associated with Myeloproliferative Disorders

A
  1. Polycythemia vera (RBCs)
  2. Essential (1o) Thrombocythemia (platelets)
  3. Chronic Myelogenous Leukemia (granulocytes)
  4. Myelofibrosis (collagen or fibrous tissue)
2
Q

Which type of disorder group has the following results: Abnormal, excessive production of erythroid, myeloid, and megakaryocytic precursors in bone marrow?

A

Myeloproliferative Disorders

3
Q

Myeloproliferative disorders are reflected in which 2 labs?  

A

peripheral blood smear & CBC results

4
Q

Myeloproliferative disorders are often caught on a CBC with what or by what on physical exam

A

irregular cell counts; splenomegaly

5
Q
What disorder group is indicative of the following presenting symptoms include: 
– Potentially  None 
– Hypertension 
– Itching 
– Burning feet 
– Bleeding 
– Thrombosis 
– An enlarged spleen (leading to abdominal fullness and discomfort)
A

Myeloproliferative Disorders

6
Q

What is a chronic myeloproliferative disorder characterized by increased Hgb concentration & increased RBC mass?

A

Polycythemia Vera (PV)

7
Q

Which condition has the following characteristics?

  • Mean age at diagnosis is 60 years old
  • Environment: exposure to high levels of radiation may increase risk
  • CBC and peripheral smear show panmyelosis: increased RBCs, WBCs, & Platelets
A

Polycythemia Vera (PV)

8
Q

Which class of polycythemia is due to low plasma volume: dehydration, burns, diuretics?

A

Relative

9
Q

Which class of polycythemia has the following characteristics?Hypoxia, seen with smoking, high altitude, lung disease

A

Secondary “Reactive Polycythemia”

10
Q

Which class of polycythemia has the following characteristics? “Malignant” hematologic disorder, not self-limiting. Treated with phlebotomy, anti-neoplastic drugs. Significant increases in all formed elements

A

Primary Polycythemia Vera

11
Q

Which myeloproliferative disorder has the following signs and symptoms?
•  Expanded blood volume & hyperviscosity lead to: weakness, headache, light-headedness, visual disturbances, fatigue, dyspnea
•  Epistaxis
•  Pruritus is frequent
•  Face may be red, retinal veins engorged
•  Hypertension
•  Hepatomegaly frequent
•  Splenomegaly in > 75% of patients

A

Polycythemia vera

12
Q

In polycythemia, oxygen content of blood _________, but oxygen saturation of tissues is ________.

A

increased; decreased

13
Q

In polycythemia, tissue hypoxia due to what?

A

increased blood viscosity, causing problems with tissue perfusion & vessel thrombosis

14
Q

In polycythemia, which phenomenon is common?

A

Raynaud’s

15
Q

Chronic cases of polycythemia results in increased ______ ______ & increased ______ _______ in an effort to decrease tissue hypoxia

A

cardiac output; capillary beds

16
Q

Which Myeloproliferative disorder has the following peripheral blood findings?
• Immature WBCs •  Immature RBCs, with marked anisocytosis and poikilocytosis: microcytes, elliptocytes, and dacrocytes may be seen •  Neutrophilia with abnormal morphology •  Thrombocytosis with abnormal morphology & if function is also abnormal then increased bleeding

A

Polycythemia vera

17
Q

Which myeloproliferative disorder has an idiopathic platelet count increase above 500,000/µL, in the absence of the features of the other myeloproliferative disorders and has the following characteristics?
•  Seen in 50-70 year olds
•  Characterized by:
•  Markedly increased platelet count
•  Bone morrow megakaryocyte hyperplasia
•  Either a hemorrhagic or thrombotic tendency.

A

Essential (1o) Thrombocythemia

18
Q

Which myeloproliferative disorder has the following diagnostic conditions?
•  Platelets 500,000 to 1,000,000
•  Normal RBC mass
•  Lack of BM fibrosis
•  Absence of dacrocytes
•  Absence of “Philadelphia Chromosome” (CML)

A

Essential (1o) Thrombocythemia

19
Q

Which myeloproliferative disorder is a condition in which bone marrow becomes fibrotic and is characterized by:

  • BM fibrosis
  • Splenomegaly
  • Normo/normochromic anemia with Dacrocytes
  • Myeloid metaplasia: Extramedullary hematopoiesis, cells formed in liver & spleen
A

Myelofibrosis

20
Q

Which myeloproliferative disorder has the following characteristics?
•  Peak incidence 50-70 years
•  Median survival 10 years from onset
•  Dx by BM biopsy showing fibrosis, marrow aspiration commonly dry •  WBC & platelet counts frequently high initially, become low as disease progresses

A

Myelofibrosis

21
Q

Which myeloproliferative disorder has the following CBC results?
• Leukoerythroblastic: Peripheral blood picture immature WBCs and RBCs.
•  RBCs: Normocytic normochromic anemia mild poikilocytosis, polychromatophilia & NRBCs, Dacrocytes
•  WBCs: Initial leukocytosis with immature neutrophils
•  Platelets: eventually thrombocytopenia

A

Myelofibrosis

22
Q

The following are other conditions that may manifest as what?

  • PV: 15-30% of cases
  • Leukemias, lymphomas, multiple myeloma
  • TB and osteomyelitis
  • Myelodysplastic Syndrome MDS
  • Exposure to benzene, X-Rays, Gamma Rays
A

a “secondary myelofibrosis” as part of their respective disease courses

23
Q

What are the following DDx conditions characteristic of?

  • Acute infection
  • Chronic inflammatory dz (RA, TB)
  • Iron deficiency anemia
  • Hemolysis
  • Cancer
  • Lymphoma
  • Splenectomy
A

DDx from causes of 2o Thrombocythemia

24
Q

Which myeloproliferative disease is the analogy of the kid that never leaves home and lives in their parent’s basement?

A

Myelodysplasia (MDS)

25
Q

Which myelodysplasic syndrome is a heterogenous group of disorders that result in ineffective hematopoiesis, and has the following characteristics?

  • USUALLY ALL 3 cell lines involved.
    1. Proliferation of abnormal clones of hematopoietic cells in BM, but with ineffective & abnormal blood cell production leading to cytopenias, with extramedullary hematapoiesis in liver and spleen resulting in splenomegaly and hepatomegaly.
    2. BM may be normal or hypercellular & contains
A

MDS

26
Q

Which myleoprolific disorder has the following signs and symptoms? Fatigue, weakness, anorexia, weight loss, abdominal fullness and may have increased bleeding & infections.

A

MDS

27
Q

Which myeloproliferative disorder has the following CBC picture?

  1. Macrocytic anemia with anisocytosis
  2. Thrombocytopenia with variations in PLT size.
  3. WBCs may be normal, high, or low
  4. May see monocytosis and up to 5% blasts in peripheral blood.
A

MDS

28
Q

The following are all examples of which type of cancer?
•  Leukemias: ALL, AML, CLL, CML
•  Hairy Cell Leukemia
•  Lymphomas –  Hodgkin’s disease –  Non-Hodgkin’s lymphoma –  Burkitt’s Lymphoma
•  Plasma Cell Dyscrasias –  Multiple Myeloma –  Waldenstrom’s Macroglobulinemia

A

Hematologic Cancer

29
Q

Which hematologic cancer has the following characteristics?
•Bone marrow overproduces abnormal WBCs •  Classified by type of cell affected (myeloid or lymphatic) –  ALL, AML, CLL, CML, Harry Cell

A

Leukemia

30
Q

Which hematologic cancer has the following characteristics?
• Uncontrolled growth of WBCs forming tumors in lymph nodes
•  Hodgkin’s, Non-Hodgkin’s, Burkitt’s

A

Lymphoma

31
Q

Which hematologic cancer has the following characteristics?
Abnormal plasma cells (produce antibodies) accumulate in bone marrow
•  Multiple Myeloma, Waldenstrom’s Macroglobilinemia

A

Myeloma

32
Q

Which cancerous condition is a process of uncontrolled, abnormal WBC growth in the bone marrow? The accumulation of cancerous cells interferes with the production of RBCs WBCs and platelets.

A

Leukemia

33
Q

Which type of cancer arises from changes within chromosomes? When chromosomes within a cell become abnormal, the cell looses its ability to control its growth and death (apoptosis)

A

Leukemias

34
Q

Which cancer is one of 10 most frequently occurring cancers. 5 year survival rate is ~55%

A

Leukemia

35
Q

The following risk factors have been implicated in which type of cancer?
–  Viruses (Hep B, EBV, HIV)
–  Exposure to Radiation
–  Environmental factors (smoking, benzenes, herbicides)
–  Genetic disorders (Down syndrome)
–  Age (most often after 60 years old)

A

Leukemia

36
Q

What is the most common malignancy in children, has a peak incidence 3-5 yrs. 80% of cases in children, second, lower peak in adults. Prognosis depends on factors present at dx: T-cell, younger age, initial WBC counts below 50,000 and quick response to chemotherapy all have a better prognosis.

A

ALL - Acute Lymphoblastic Leukemia

37
Q

Which condition is found 80% of adult acute leukemias. Mean age: 65 Poor prognosis, especially patients > 50 years

A

AML - Acute Myeloblastic Leukemia

38
Q

Which condition is seen as 20% of all leukemia, both sexes affected, common btw. ages of 20-50, rare in childhood, and has a poor prognosis?

A

CML - Chronic Myelogenous Leukemia

39
Q

Which condition is 30% of all leukemia, usually middle age to elderly, twice as common in men than women, and has an average survival of 3-7 years after dx?

A

CLL - Chronic Lymphocytic Leukemia

40
Q

What diagnostic criteria is describe below?
- Blood examination
–  PLT Low in 20-30%
–  WBC 20-150,000/ml
– Increased mature lymphocytes
– Smudge cells •  immature lymphocytes with increased fragility partially broken down during slide preparation
–  Dx is made from a sustained ABS lymphocytosis of > 5000/ml Bone Marrow infiltrated with small lymphocytes

A

Chronic Lymphocytic Leukemia

41
Q

What condition has the following diagnostic condition?
• Blood examination
–  Anemia in 90%
–  Thrombocytopenia in 80% of cases
–  WBC variable, usually elevated –  Lymphoblasts common in peripheral smear
•  Bone Marrow morphology
–  greater than 30% blasts in marrow usually means leukemia
–  Suppression of normal hematopoiesis
•  Strongly reactive to special stain: Periodic acid-Schiff

A

Acute Lymphoblastic Leukemia

42
Q

Which condition has the following diagnostic criteria?
•  Blood examination
–  90% Anemia
–  Thrombocytopenia
–  WBC variable, usually increased
–  Myeloblasts common in peripheral blood
•  Bone marrow morphology
•  Greater than 30% blasts
•  Strongly reactive to special stain: Sudan black B
•  Genetic studies are important in classification and prognosis

A

Acute Myeloiblastic Leukemia

43
Q

Which condition has the following diagnostic characteristics?
•  Auer rods: Found in Blasts. Small, rod-shaped cytoplasmic elements. Thought to be abnormal lysosomes.
Increased segmented neutrophils, myelocytes and metamyelocytes are seen in the peripheral blood.

A

Acute Myeloid Leukemia

44
Q

Which condition has the following diagnostic characteristics?
•  Blood examination •  Asymptomatic WBC

A

Chronic Myelocytic Leukemia

45
Q

What are a heterogeneous group of neoplasms that arise in the lymphatic and reticuloendothelial (RE) systems?

A

Lymphomas

46
Q

What are the two major types of lymphomas? What is one other rare type?

A
  1. Hodgkin’s disease
  2. Non-Hodgkin’s lymphoma
  3. Burkitt’s Lymphoma
47
Q

Which type of lymphoma has the following characteristics?
•  Reed-Sternberg cells: Dx is by lymph node biopsy showing them
•  There can be a slight-to-moderate neutrophilia
•  Lymphocytopenia can occur early & become pronounced with advanced disease
•  Eosinophilia is present in 20% of patients
•  Thrombocytosis can be present
•  Increased ESR reflect active disease
•  Microcytic hypochromic anemia in advanced dz

A

Hodgkin’s disease

48
Q

Which type of lymphoma has the following characteristics?
•  Bimodal age distribution, peaks age15-34 & after age 60
•  Spreads to contiguous lymph nodes
•  Etiology unknown, but twin studies have shown genetic component. •  Hx of EBV, HIV, occupations (woodworkers)
•  Pain in affected areas after consuming alcohol; fever, night sweats; intense pruritis is an early sign

A

Hodgkin’s disease

49
Q

Which type of lymphoma has the following characteristics?
•  Clonal proliferation of lymphoid cells in lymph nodes, bone marrow, tonsils, spleen, liver, or GI
•  Etiology unknown.
•  May be due to HTLV-1 Human T-cell Lymphoma Virus 1
•  Anemia present in 33% initially, most develop it as disease progresses with non-contiguous spread
•  Leukemic phase develops in 20-40% of lymphocytic lymphomas
•  Dx can only be made via lymph node biopsy

A

Non-Hodgkin’s lymphoma NHL

50
Q

According to the Ann Arbor Staging System, which stage has single lymph node region or single extralymphatic organ or site?

A

Stage I

51
Q

According to the Ann Arbor Staging System, which stage has > 2 lymph node regions on same side of diaphragm or with limited, contiguous extra lymphatic tissue involvement?

A

Stage II

52
Q

According to the Ann Arbor Staging System, which stage has both sides of diaphragm involved, may include spleen or local tissue involvement?

A

Stage III

53
Q

According to the Ann Arbor Staging System, which stage has multiple/disseminated foci involved with >1 extra-lymphatic organs (i.e. bone marrow)

A

Stage IV

54
Q

Which type of lymphoma is characterized below?
•  Highly undifferentiated B cell lymphoma
•  Can involve sites other than the lymph nodes & reticuloendothelial system
•  Most common in Central Africa
•  Epstein Bar Virus: Associated with history of infection

A

Burkitt’s lymphoma

55
Q

What are a group of clinically and biochemically diverse diseases characterized by the proliferation of one clone of plasma cells normally engaged in immunoglobulin (Ig) production. They are typified by the presence of a monoclonal Ig or polypeptide subunit in serum or urine.

A

Plasma Cell Dyscrasias (PCDs)

56
Q

What are the two main forms of Plasma Cell Dyscrasias (PCDs)?

A
  1. Multiple Myeloma

2. Waldenstrom’s Macroglobulinemia

57
Q

Which condition has unexplained bone pain (back, thorax), renal failure, & recurrent bacterial infections most common? May see pathological fractures in vertebrae & anemia.

A

Multiple Myeloma Symptoms

58
Q

Which condition’s symptoms are described below?

  • Most are asymptomatic
  • May have indications of “hyperviscosity syndrome” of fatigue, bleeding from skin and mucous membranes, weakness, visual disturbances & headaches
  • Also Raynaud’s phenomenon.
A

Waldenstrom’s Macroglobulinemia Symptoms

59
Q

Which condition is described below?
•  Progressive neoplastic dz
•  Plasma cell tumors in BM
•  Increased production of Specific monoclonal Ig:G,A,D,E
•  Urinary Bence Jones protein Free monoclonal κ or λ light chains Light chain = higher bone lytic lesions and associated hypercalcemia & renal failure

A

Multiple Myeloma

60
Q

What is the C.R.A.B. mneumonic for multiple myeloma?

A

C: hyperCalcemia
R: Renal failure
A: Anemia
B: Bone lesions

61
Q

Which condition fits the diagnostic criteria below?
•  Anemia: normocytic,normochromic
•  RBCs form rouleaux due to increased viscosity from abnormal proteins
•  WBC & platelets counts usually normal
•  ESR: often markedly elevated, > 100 mm/hr

A

Multiple Myeloma

62
Q

Which condition fits the diagnostic criteria below?
•  55% produce IgG and 20% produce IgA.
•  Total protein, BUN, creatinine, uric acid elevated
•  Hypercalcemia in 1/3 of patients
•  40% Bence Jones proteinuria, (κ or λ light chains) urine need sulfosalicylic acid test to detect.
•  Serum protein electrophoresis : increased monoclonal Ig protein, or “M protein” in 80%

A

Multiple myeloma

63
Q

Which condition fits the diagnostic criteria below?
•  Clonal expansion of plasma cells that normally synthesize & secrete IgM
•  Macroglobulinemia resembles lymphomas & many of its clinical manifestations are due to large amounts of circulating macroglobulin
•  Many patients have a hx of Raynaud’s phenomenon
•  Recurrent bacterial infections are a major problem.

A

Waldenstrom’s Macroglobulinemia

64
Q

Which condition fits the diagnostic criteria below?
•  Moderate anemia
•  Marked rouleaux formation & very high ESR
•  Leukopenia, relative lymphocytosis and thrombocytopenia occasionally occur
•  Cryoglobulins, rheumatoid factor, and cold agglutinins may be present.
•  Relative serum viscosity is usually > 4.0 (normal 1.4-1.8)

A

Macroglobulinemia