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Hematology > Hematology Exam 5 > Flashcards

Hematology Exam 5 Flashcards

1
Q

Briefly describe SCID

A

Congenital nonmalignant leukocyte disorder
Marked decrease in circulating T cells, poorly functioning B cells, hypogammaglobulinemia

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2
Q

What is the gamma chain deficiency (X-linked SCID)

A

Caused by mutation in the IL2RG gene on the X chromosome resulting in a severe decrease in T and NK cells, adequate number of B cells, but they are dysfunctional

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3
Q

What is the adenosine deaminase (ADA) deficiency SCID?

A

Mutation on the ADA gene on chromosome 20 resulting in decreased T, B and NK cells

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4
Q

What is Wiskott-Alrich (WAS) Syndrome?

A

Congenital nonmalignant WBC disorder
rare X-linked disease due to mutation in WAS gene characterized by decreased T cells and dysfunctional Tcells, Bcells, NK cells, neutrophils, and monocytes

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5
Q

What is 22Q11 Syndrome?

A

Absence/decreased size of thymus due to a missing part of chromosome 22 characterized by decreased T cells

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6
Q

What is Bruton Tyrosine Kinase Deficiency?

A

Congenital nonmalignant WBC disorder
X-linked agammaglobulinemia due to mutation of BTK gene - no serum antibodies and profoundly decreased or absent B cells

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7
Q

What is Chediak-Higashi Syndrome?

A

Congenital nonmalignant WBC disorder
Rare autosomal recessive disease of immune dysregulation due to a mutation in the CSI LYST gene
- associated with albinism and giant lysosomal granules in WBCs

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8
Q

What are LADs?

A

Leukocyte adhesion disorders - inability of neutrophils and monocytes to move from circulation to the site of inflammation

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9
Q

What is Schwachman-Diamond Syndrome (SDS)?

A

A defect in leukocyte motility caused by mutation in SBDS gene on chromosome 7

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10
Q

What is Chronic Granulomatous Disease?

A

Decreased ability of neutrophils to undergo a respiratory burst resulting in life-threatening catalase-positive bacterial and fungal infections

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11
Q

What is WHIM syndrome?

A

Warts, hypogammaglobulinemia, infections, myelokathexis (low numbers of circulating neutrophils because they are all in the BM) due to mutation on the CXCR4 gene

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12
Q

What are the two types of SCID (Severe Combined Immunodeficiency)

A

Gamma chain deficiency (X-linked)
ADA deficiency

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13
Q

What cells are seen in Pelger-Huet anomaly?

A

Pelger-Huet cells which have decreased segmented neutrophils (most of them are only bi-lobed and have dense chromatin) the nucleus looks like dumbbells

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14
Q

What type of cells are seen in Chediak-Higashi Syndrome?

A

Granulocytes/monocytes/lymphocytes with giant lysosomal granules

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15
Q

What type of cells are seen in neutrophil hypersegmentation?

A

Hypersegmented neutrophils –> >5 lobes on neutrophils

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16
Q

What type of cells are seen in Alder-Reilly anomaly?

A

large, darkly staining metachromatic cytoplasmic granules called Reilly bodies containing partially digested mucopolysaccharides

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17
Q

What is May-Hegglin Anomaly and what type of cells do you see with this disease?

A

Characterized by:
- thrombocytopenia
- Giant PLTs
- Large Dohle body-like inclusions
See dohle bodies on WBCs

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18
Q

What are lysosomal storage diseases?

A

Enzyme deficiencies that result in a buildup of undigested substrates within lysosomes called mucopolysaccharides

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19
Q

What are sphingolipidoses? What are the examples of them?

A

Disorder in which lipid catabolism is defective; such as Gaucher disease and Neimann-Pick disease

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20
Q

What are Gaucher cells?

A

distinctive macrophages that look like onion skin seen in Gaucher disease

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21
Q

What nonmalignant WBC disorder has a highest rate of incidence in Ashkenazi Jews?

A

Gaucher disease

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22
Q

What is Niemann-Pick Disease and what cells are seen with this disease?

A

Accumulation of fat in lysosomes that causes impaired organ function - Foam cells AKA Niemann-Pick cells and sea blue histiocytes

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23
Q

How are the different ways you can differentiate between a leukemoid reaction and a chronic myeloid leukemia?

A
  • LAP score (High in LR and decreased in CML)
  • Genetics (Normal in LR and positive for philadelphia chromosome/BCR-ABL in CML)
  • Hemoglobin (Normal in LR and anemia in CML)
  • Eos and Basos (Normal in LR and elevated in CML)
  • Neutrophils (toxic gran/dohle seen in LR and pseudo-pelger-huet in CML)
  • Platelets (normal in LR and abnormal in CML)
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24
Q

Leukoerythroblastosis

A

Increase in immature WBCs/RBCs - Presence of immature neutrophils, nRBCs, and dacrocytes

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25
Q

Agranulocytosis

A

Extremely low number of granulocytes

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26
Q

What is the most common cause of acquired neutropenia?

A

Medications (drug-induced)

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27
Q

Neutropenia

A

decreased neutrophils could be due to medications such as anticancer drugs

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28
Q

Neutrophilia

A

Increased neutrophils

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29
Q

Eosinophilia

A

Increased eosinophils seen with helminth infections or allergic reactions

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30
Q

Eosinopenia

A

Decreased eosinophils

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31
Q

Basophilia

A

Increased basophils associated with CML, allergic rhinitis, hypersensitive rxns

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32
Q

Monocytosis

A

Increased monocytes due to infections or postsplenectomy

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33
Q

Monocytopenia

A

Decreased monocytes due to anemia or steroid therapy

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34
Q

Describe toxic granulation and associated diagnosis

A

Dark, blue-black cytoplasmic granules in neutrophils associated with inflammation, infection, G-CSF

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35
Q

Describe Dohle bodies and associated diagnosis

A

Intracytoplasmic pale blue/round body adjacent to cellular membrane associated with infection, G-CSF, pregnancy, burns

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36
Q

Describe cytoplasmic vacuoles and associated diagnosis

A

Clear circular areas in cytoplasm that may contain an organism (rare) associated with bacterial infections

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37
Q

Intracellular organisms

A

bacteria that grow in white blood cells

38
Q

Ehrlichia and Anaplasma - what are they and where do each of them grow

A

small intracellular bacteria transmitted by ticks to humans
Ehrlichia grows as a cluster in monocytes
Anaplasma grows as a cluster in neutrophils

39
Q

What is pyknotic nuclei?

A

Very dark chromatin indicating cell is going to die

40
Q

What is necrotic nuclei?

A

Rounded nuclear fragments with no chromatin pattern found in dead neutrophils

41
Q

What do increased numbers of pyknotic and necrotic cells suggest?

A

An extended amount of time has elapsed between blood collection and blood film preparation

42
Q

What is cytoplasmic swelling?

A

Variation in neutrophil size due to swelling of the cytoplasm or increased adhesion to the glass slide

43
Q

morphological features of reactive monocyte

A

Thin/band-like segmented nuclei and irregular cytoplasmic borders that take on the shape of the RBCs surrounding it

44
Q

Reactive lymphocyte morphology

A

Nucleoli may be visible
Increase in basophilic cytoplasm
Cytoplasm is indented by surrounding RBCs

45
Q

Reactive vs Abnormal lymph

A

Reactive is used to refer to benign etiology
Abnormal is used to refer to malignant/clonal etiology

46
Q

Morphology of a plasmacytoid lymph and what does its presence indicate in high numbers?

A

Has some features of plasma cells - very blue cytoplasm and if seen in high numbers suspect lymphoma or Waldenstrom’s Macroglobulinemia

47
Q

What is the causative agent of infectious mononucleosis?

A

Epstein Barr Virus

48
Q

What cells does Epstein Barr Virus infect?

A

Primarily infects B lymphs, so most circulating reactive lymphs are T cells

49
Q

What population of people does epstein barr virus typically infect?

A

Mostly seen in young adults age 15-24 years

50
Q

Lab findings of those with EBV

A

Absolute lymphocytosis with up to 50% or more reactive lymphs; Positive for heterophile antibodies

51
Q

Clinical manifestations of EBV

A

Sore throat, dysphagia, fever, chills, cervical lymphadenopathy, fatigue, headache

52
Q

Define and describe an MPN

A

clonal hematopoietic disorder that result in expansion, excessive production, and accumulation of mature RBCs/WBCs and PLTs that are predominantly chronic - may progress into acute leukemias

53
Q

What are the four major MPNs?

A

Chronic myeloid leukemia (CML)
Polycythemia Vera (PV)
Essential Thrombocytopenia (ET)
Primary myelofibrosis (PMF)

54
Q

What do EV, PV, and PMF have in common that is NOT associated with CML?

A

EV/PV/PMF are genetically related based on the presence of the JAK2 mutation and the ABSENCE of the philadelphia chromosome or BCR-AB1 fusion gene

55
Q

What is ET associated with?

A

Thrombocytosis

56
Q

What is PV associated with?

A

Erythrocytosis

57
Q

What is MPF associated with?

A

Neutrophilia, left shift, fibrosis

58
Q

What is CML associated with?

A

PRESENCE of the Philadelphia chromosome and/or the BCR-ABL1 fusion gene

59
Q

What is REQUIRED for a CML diagnosis?

A

Presence of the philadelphia chromosome or the BCR-ABL1 fusion gene

60
Q

MPNs may transform into ______ or _______.

A

AML or ALL (leukemic transformation)

61
Q

What is the Philadelphia chromosome?

A

t(9;22) that produces a fusion gene called BCR-ABL1 that must be present to confirm a CML diagnosis

62
Q

Where is the ABL1 gene found? BCR gene?

A

ABL1: chromosome 9
BCR: chromosome 22

63
Q

How does the presence of Philly chromosome and BCR-ABL1 fusion gene lead to the findings seen in CML?

A

Increased clonal proliferation of myeloid cells secondary to a reduction in protein regulators

64
Q

Describe CML (cell line most affected, clinical features, pathogenic mechanism, notable lab findings, treatment)

A

Affects myeloid cell line (increased immature neutrophils)
Clinical features: anemia, bleeding, splenomegaly, frequent infection
Lab findings: Increased basophils, eosinophils, neutrophils, platelets, decreased LAP score
Treatment: Imatinib mesylate (Gleevec)

65
Q

What is Imatinib mesylate?

A

Gleevec; a tyrosine kinase inhibitor

66
Q

PV cells affected

A

Increased RBCs, granulocytes, and PLTs

67
Q

PV pathogenic mechanism

A

Neoplastic clonal stem cells function independently of erythropoietin for cell growth (erythropoietin independent erythropoiesis –> make RBCs without erythropoietin caused by mutated JAK2 gene

68
Q

4 major criteria to diagnosis PV

A

Elevated HGB, HCT, or red cell mass
BM showing panmyelosis
Identificiation of the JAK2 V617F mutation
Low serum EPO levels

69
Q

What cell line is increased with ET?

A

Increased megakaryopoiesis and thrombocytosis

70
Q

What is the most common cause of death in ET patients?

A

Thrombosis and bleeding

71
Q

What is the least common, but most aggressive MPN?

A

primary myelofibrosis (PMF)

72
Q

What separates PMF from other MPNs?

A

Increase in circulating CD34+ cells (HSCs)

73
Q

What is the pathogenic mechanism of PMF?

A

Mutation in 1 of 3 genes that affect the JAK-STAT pathway: JAK2 (V617F), CALR, or MPL

74
Q

What is PMF characterized by?

A

Overproduction of collagen that leads to replacement of hematopoietic tissue leading to pancytopenia

75
Q

What is seen in the peripheral blood and bone marrow of PMF?

A

Peripheral blood: dacrocytes and leukoerythroblastosis

Bone barrow: DRY TAP –> hypocellular

76
Q

Describe MDSs as a group

A

A group of acquired clonal hematologic disorders characterized by progressive cytopenias and defects in erythroid, myeloid, and/or megakaryocytic maturation (DECREASED CELLS!)

77
Q

What 2 morphological findings are common to all types of MDS?

A

Presence of progressive cytopenias despite cellular BM, and dyspoiesis in one or more cell lines

78
Q

What is the most common morphological finding in the peripheral blood in dyserythropoiesis?

A

Oval macrocytes

79
Q

What abnormal cell morphology is seen in dysmyelopoiesis?

A

abnormal granulation and cell features; ring shaped nuclei

80
Q

What does dysmyelopoiesis indicate?

A

Nuclear-cytoplasmic asynchrony

81
Q

What abnormal cells are seen in the bone marrow of dyserythropoiesis?

A

Nuclear bridging, ringed sideroblasts, RBC precursors with more than 1 nuclei

82
Q

What abnormal cells are seen in dysmegakaryopoeisis?

A

Giant platelets, abnormal PLT granulation

83
Q

What is MDS with single lineage dysplasia?

A

Dysplasia present in at least one myeloid lineage with one type of cytopenia

84
Q

What is MDS with multilineage dysplasisa?

A

Dysplasia in two or more myeloid cell lines with one of more cytopenias

85
Q

What is MDS with ringed sideroblasts?

A

Mutation of the gene SF3B1; ring sideroblasts present (iron containing mitochondria)

86
Q

What is MDS with excess blasts?

A

Trilineage cytopenia with presence of Auer rods

87
Q

What is MDS with DEL 5Q

A

The only WHO recognized MDS with defining cytogenetic abnormality that predominantly affects women - anemia without other cytopenias and hypolobulated megakaryocytes, NO auer rods

88
Q

What Is MDS unclassifiable?

A

Only one cell line is dysplastic, but all cell lines are decreased

89
Q

What are the MDS/MPNs?

A

Chronic myelomonocytic leukemia
Atypical chronic myeloid leukemia
Juvenile myelomonocytic leukemia
Refractory anemia with ring sideroblasts and thrombocytosis

90
Q

What treatment is used for MDS/MPNs?

A

Lenalidomide

91
Q

What are MDS/MPNs?

A

Myeloid neoplasms that share characteristics of BOTH MDS and MPNs

Hematology (10 decks)

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