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Hematology II Test 2 > General Information > Flashcards

General Information Flashcards

1
Q

This is a Nonmalignant Leukocyte disorder that is autosomal dominant characterized by hyposegmentation of the granulocytes (most apparent in neutrophils).

A

Pelger Huet Abnormality

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2
Q

Pseudo Pelger-Huet cells are found in patients

A

during or after chemotherapy

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3
Q

Does Pelger Huet abnormality cause disease?

A

No

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4
Q

This is a Nonmalignant Leukocyte disorder that is autosomal dominant and clinically insignificant. It is characterized by hypersegmentation of the neutrophil.

A

Hereditary Hypersegmentation Anomally

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5
Q

How can you differentiate between Hereditary Hypersegmentation and Megaloblastic Anemia?

A

Megaloblastic Anemia will have oval macrocyts, hypersegmentation, and an MCV ? 120.

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6
Q

This Nonmalignant Leukocyte Disorder is very rare and characteized by cells that are a mirror image with axel symmetry. It is aquired during malignancies and chemotherapy.

A

Twinning Anomaly

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7
Q

This Nonmalignant Leukocyte disorder can be caller “drumstick” or “chicken leg”, and is believed to be a reminant of an inactivated X chromosome in women.

A

Nuclear Extrusion anomaly

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8
Q

Increased numbers of these cells are linked to genetic problems

A

Nuclear Extrusion anaomaly cells

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9
Q

This disorder is characterized by large or dominant primary granules. 1 of 3 toxic changes. Neutrophils can look like eosinophils, and they are clinically significant. Watch for staining errors.

A

Toxic granulation

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10
Q

This disorder is a clinically significant reminant of phagocytosis. Is is 2/3 of the toxic changes and is formed by incomplete digestion and degridation of bacteria and fungi.

A

Vaculolization

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11
Q

This leukocyte disorder occurs when neutrophils accumulate RNA. 3/3 of the toxic changes. Can be in bands, segs, monos, ect.

A

Dohle Bodies

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12
Q

Dohle Bodies are caused by a stimulating event, such as

A 
Burns
Infections
Surgery
Pregnancy
Use of Granulocyte Monocyte Colony Stimulating Factor (GMCS)
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13
Q

These 3 toxic changes are always clinically significant and always reported.

A

Toxic Granulation
Vacuolization
Dohle Bodies

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14
Q

This disorder has cells that have small pyknotic chuncks, characteristic of a neutrophil that dies and falls apart. Low numbers are NOT clinically significant, but large numbers are.

A

Necrobiosis

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15
Q

This is a recessive anomally characterized by decreased mucoplysaccharide. Can be found in granulocytes. Can barely see nucleus because of prominent, heavy granulation.

A

Alder-Reily Anomally

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16
Q

This anomally can be seen in Hunters or Hurlers syndrome

A

Alder-Reily Anomally

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17
Q

This is a rare autosomal recessive disease characterized by giant, lysosomal granules in cells. It is genetic and fatal (bone marrow transplant sometimes). Patient has recurrent bacterial infections.

A

Chediak-Higashi Syndrom

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18
Q

This rare autosomal dominant disorder has large Dohle-like bodies which are RNA fibrils. Often accompanied by thrombocytopenia but many patients are asymptomatic. Patients are at risk for infections and mucutaneous hemorrhage. Bleeding episodes reported.

A

May-Hegglin Anomally

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19
Q

This disease is familial and heterozygous. Cells develop granulomas when they cannot complete process of phagocytosis. Reoccurent bacterial/fungal disease.

A

Chronic Granulomatous Disease of Childhood

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20
Q

How is Granulomatous Disease of Childhood diagnosed?

A

Nitoblue tetrazolium (NBT): measures respiratory burst needed for phagocytosis

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21
Q

Gangliosidosis, Tay-Sachs, Fabry, Aquired hyperlipidemia, Gauchers, and Neimann Picks disease are all due to

A

abscence of a particular enzyme which leads to accumulatied products

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22
Q

This is the most common storage disease and is autosomal recessive. It affects macrophages. Enzyme deficiency leads to build up of glucocerebroside (lipid).

A

Gaucher Disease

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23
Q

Type 1 Gauchers is found in and characterized by

A

Ashkenazi- No CNS involvement, symptoms asymptomatic to sever

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24
Q

Type 2 Gauchers is found in and characterized by

A

Acute neuropathic- CNS involvement, much more sever then type 1

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25
Q

Type 3 Gauchers is

A

an intermediae stage

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26
Q

Pseudo Gaucher cells can be found in this disorder

A

Myeloproliferative disorders (CML: chronic myologenour leukemia)

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27
Q

This autosomal recessive disease affects enzyme storage in the macrophage. Accumulation of sphingomyelin in spleen, lungs, liver, bone marrow, and sometimes the brain.

A

Niemann Pick Disease

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28
Q

What are the types of Niemann Pick Diseas?

A 
Type A: Ashkenazim, acute neuropathy
Type B: chronic, no CNS
Type C: progressive deterioration (including dementia), vegitative state, death at 5 or 6
Type D: Same as C, Nova Scotian
Type E: adult non-neuropathic
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29
Q

Very large nucleoli are found in the blast stages of

A

leukemias

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30
Q

This leukemia is found in children between 2-10 years old (second peak at 40 years old), prognosis based on age and lymphoblast load. Lab findings are peripheral blood lymphoblasts > 20-30 x 10^9/L, hepatoslenomegaly (big spleen) and lymphodenopathy (swollen glands). Find small blasts wit indistinct nucleoli, abd karge blasts with prominint nucleoli.

A

Acute Lymphoblastic Leukemia (ALL)

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31
Q

What is the treatment for Acute Lymphoblastic Leukemia (ALL)?

A

Chemotherapy AND a spinal tap

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32
Q

What is the prognosis for children and adults of Acute Lymphoblastic Leukemia (ALL)?

A

Children: 90% remission, 60% cure
Adults: 68-91% remission, 25-41% cure

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33
Q

These feature indicate which type of ALL?
CALLa pos
HLA-Dr and TdT pos
Neg (CIg/SIg)

A

Immature B cell ALL
CALLs pos: prescence of CD10
Neg internal or surface cytoplasmic immunoglobulin (CIg/SIg)

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34
Q

These feature indicate which type of ALL?
CALLa neg
Pos CIg
Neg SIg

A

Pre-B cell ALL
CALLa neg: abscence of CD10
Pos internal cytoplasmic immunoglobulin (CIg)
Neg surface cytoplasmic immunoglobulin (SIg)

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35
Q

This type of ALL has the best prognosis because of anti-CD20 (rituximab) therapy.

A

Pre-B cell ALL

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36
Q

These feature indicate which type of ALL?
May be TdT pos
Teen males w/ mediastinal mass, increased blast counts, meningeal involvement
Poor prognosis

A

T cell ALL

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37
Q

These feature indicate which type of ALL?
Pos SIg: kappa and lamda chains
VERY poor prognosis

A

B cell ALL

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38
Q

Treatment for children with ALL

A

Methotrexate- pregnant women cannot be around methotrexate

39
Q

Chronic leukemias show _____, while acute leukemias show ______

A

Chronis leukemias show maturation, while acute leukemias show blasts and pros

40
Q

This leukemia is found in children under 1 (second peak around 40years). FAB system of classification. Decreased production of normal bone marrow elements. Prescence of many myeloblastsa in peripheral blood.

A

Acute Myelogenous Leukemia

41
Q

FAB classification is only for the ____ leukemias

A

acute

42
Q

This stains RNA in the ribosomes of the endoplastic reticulum. Used to stain reticulocytes.

A

Supre vital stains

43
Q

This stains primary granules and makes Auer rods strongly positive. Used in all stages of granulocyte development including blasts (lymps will be neg, monos weakly positive)

A

Myeloperoxidase (MPX)

44
Q

This stains lipids and has a staining pattern similar to MPX.

A

Sudan Black B (CBB)

45
Q

This stain differentiates myloblasts and neutrophils from monocytes. Cells with nonspecific granules are usually negative (granulocytes and lymphs) which monocytes are positive.

A

Esterases (hydrolyzes esters)

46
Q

This stain is used for ALL’s and erythroleukemia. Granulocytes are positive and get more so as they mature.

A

Periodic Acid Schiff (PAS)

47
Q

This stain is used for megakaryoblastic leukemia (monoclonal or polyclonal antibodies)

A

Factor VII antibodies

48
Q

This stain differentiates chronic myelogenous leukemia (neg) from leukemoid reactions in sever infections (positive)

A

Leukocyte Alkaline Phosphatase (LAP)

49
Q

This stain finds Hairy cell leukemia because these cells will remain positive in the prescence of tartate acid.

A

Acid Phosphatase (tartrate resistant)

50
Q

Leukemias are in the ____, while lymphomas are in the _____.

A

Leukemias are in the blood and lymphomas are in the lymph nodes.

51
Q

Leactive lymphs can be seen with any

A

virus

52
Q

This can be cause by the Epstein-Barr virus and most people recover spontaneously. Can be spread by saliva (the kissing diseae) and it affects children and young adults.

A

Infectious Mononucleosis

53
Q

People with Infectious Mononucleosis caused by the Epistein-Barr virus may develop

A

Hodgkins lymphomas
Chronic Fatigue syndrom
Burkitts lymphomas

54
Q

How do you test for Infectious Mononucleosis?

A

Kit adaptations of the Paul-Bunnel Test

Heterophile antibody

55
Q

What lab finding will be present in Infectious Mononucleosis?

A 
Decrease neutophils (%)
Increased absolute lymph counts
56
Q

Myelodysplastic Syndromes are charactized by

A

AQUIRED cytopenias in the peripheral blood caused by defects in erythroid, myeloid, or megakaryocytic maturation.

57
Q

This is a primary Myelodysplastic Syndromes

A

De novo

58
Q

This is a therapy related Myelodysplastic Syndromes

A

t-MDS (sequella to chemotherapy or radiation)

59
Q

Myelodysplastic Syndromes affect people over the age of

A

70

60
Q

Myelodysplastic Syndromes may be the result of

A

environmental exposure

61
Q

This may be responsible for ineffective hematopiesis in Myelodysplastic Syndromes

A

Disruption of apoptosis

62
Q

What are the FAB classifications of Myeloblastic Disorders (bone marrow issues)

A
  1. Refractory anemia (does not respond to treatment)
  2. (RARS) Refractory anemia with ringed sideroblasts
  3. (RAEB) Refractory anemia with excess blasts
  4. (CMML) Chronic myelomonocytic leukemia
  5. (RAEB-t) Refractory anemia with excess blasts in transformation
63
Q

In Myeloblastic Syndromes, chromosome abnormalities are found in ____% of De novo, and ___% of t-MDS patients

A

40-70% De Novo

90% t-MDS

64
Q

What are 2 examples of myeloblastic syndrom chromosome abnormalities?

A

5q syndrom- chromosome deletion in women over 50 (stable disease)
Monosomy 7- 1 chromosome when you should have 2

65
Q

What is the most common sequelle of myeloblastic syndomes?

A

Acute leukemia

66
Q

What causes death in myeloblastic syndroms?

What is the life expectancy?

A 
Actue leukemia
Sever cytopenia (Bleeding)
Bone Marrow Failure
Organ Failure
1-6 year life expectancy
67
Q

What is the IPSS System?

A

A way of providing information to patients and doctors; guide to treatment for myeloblastic syndroms and prognosis

68
Q

In the lymph nodes, follicles contain

A

B cells

69
Q

In the lymph nodes, the cortex contains

A

T and B cells

70
Q

In the lymph nodes, the medulla contains

A

plasma cells

71
Q

Lymph node neoplasms affect areas of the lymph nodes. What are the four patterns associated with lymph node neoplasms?

A
  1. Follicular
  2. Paracortical
  3. Sinusoidal
  4. Mixed
72
Q

What are the characteristics of follicular hyperasia?

A

Seen in children/adolescents
Reaction to infection
In adults: autoimmune disorders (RA, SLE), syphillus and early HIV

73
Q

What are the characteristics of the paracortical pattern (around the cortex)?

A

Viral infections
Drug reactions
Chronic skin disease
IgM made in paracortical cortex

74
Q

What are the characteristics if the simusoidal pattern (in the sinusis)?

A

Lymph nodes draining limbs
Abdominal organs
Inflammatory lesions
Malignancies

75
Q

What are the characteristics of mixed patterns?

A

Toxoplasma gondii

76
Q

What is the term for a tumor of the lymph nodes? Risk factors include autoimmune disease and a comprimised immune system.

A

Lymphomas

77
Q

Exposures to chemicals and herbicides MAY predispose to

A

lymphoid neoplasms

78
Q

Mature B Cell lymphomas include

A

CLL/SLL
Hairy Cell
Plasma Cell
Burkitt lymphoma/leukemia

79
Q

Mature T Cell lymphomas include

A

Leukemis/Disseminated
Cutaneous (mycosis fungoids, Sezary syndrom)
Other extranodal
Nodal
Neoplasm of Uncertain Lineage and State of Differentioation

80
Q

Neoplasm means

A

new growth

Could be tumor or just a bunch of cells growing

81
Q

Lymphomas ofte start as

A

lymphomas

82
Q

A symptom of B cell lymphomas is

A

night sweats

83
Q

Chronic Lymphocytic Leukemia (CLL) and Small Lymphocytic Lymphoma (SLL) are the same disease but

A

Chronic Lymphocytis Leukemia is found in the peripheral blood

Small Lymphocytis Lymphoma (SLL) has moved to the lymph nodes

84
Q

Characteristics of Chronic Lymphocytic Leukemia (CLL) and Small Lymphocytic Lymphoma (SLL)

A 
Small, dense , mature Lymphs
Smudge cells
Lymphocytosis 
Incurable
65 and over
85
Q

Characteristics of Hairy Cell Leukemia

A 
Small B lymphs
Bone Marrow and red pulp of spleen
Median age 55
Splenomegaly and pancytopenia
Interferon- remission
86
Q

This stain can be used to see Hairy Cell Leukemia

A

Tartrate resistant acid phosphatas stain

87
Q

Plasma cells are B cells which are actively making

A

antibodies (that is why they are so basophilic)

88
Q

Monoclonal proliferation of plasma cells (formally Multiple Myeloma) is caused by

A

1 cell that cannot figure out how to stop dividing

89
Q

Characteristics of Monoclonal proliferation of plasma cells (formally Multiple Myeloma)

A

Lots of plasma cells
Lytic bone lesions (intense bone pain/fragile bones)
Monoclonal gammopathy

90
Q

This will be found in the urine of patients with Monoclonal proliferation of plasma cells (formally Multiple Myeloma)

A

Bence-Jones proteins (coagulate in heat, relax when cooled)

91
Q

Characteristics of Burkitt Lymphoma (can be a leukemia)

A 
"Starry Sky" lymphocytes
Children and Immunodeficient
Endemic and Sporadic
Can result from Epstien Barr infection
Classed as a medical emergency
92
Q

Bi-nucleated cells in the bone marrow are called

A

Reid- Sternberg cells

93
Q

Characteristics of Hodkin Lymphoma

A

Reed-Sternberg cells (bi-nucleated)
Bi-modal distribution (15-34 yrs, >54 yrs)
Subtypes (most agressive lymphocyte depleted in HIV)
Stages: Ann Arbor System for malignant lymphomas
80-90% cure rate (depending on stage)
High risk for secondary malignancy

Hematology II Test 2 (3 decks)

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