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WBC disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

Normal hematopoiesis

A
  • common myeloid progenitor
    1. myloblast- granulocyte
    2. Erythroblast- erythrocyte
    3. monoblast- monocyte
    4. megakaryoblast- megakaryocytes- platelet
  • Common lymphoid progenitor
    1. Blymphoblast- B cell
    2. lymphoblast- T cell
    3. NK-blast- NK cell
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2
Q

Gener principles of hematopoiesis;

A
  • hematopoiesis begins in intrauterine life in yolk sac- mesoderm- liver- bone marrow
  • By birth, bone marrow throughout the skeleton is hematopoietically active
  • by puberty , limited to axial skeleton
  • If demand is high, stem cells can migrate out of bone marrow to attempt blood cell production(extra medullary hematopoiesis)
  • Myeloid cells: bone marrow- blood- tissues
  • Lymphoid cells: bone marrow- blood- lymphoid organs- blood - tissues
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3
Q

What is Leukocytosis?

A
  1. refers to increase in the number of white cells in the blood
    - Can be reactive or neoplastic
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4
Q

Etiopathogenesis of leukocytosis:

A
  1. size of precursor pool in bone marrow increases due to increased production -causes from chronic infection/ inflammation/ neoplastic
  2. increased rate of release of cells from bone marrow - acute inflammation
  3. Decreased proportion of cells adherent to vessel walls (exercise, catecholamines)
  4. Decreased rate of extravasation of cells from blood vessel into tissue- glucocorticoids
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5
Q

Neutrophil:

A

Acute bacterial infection, sterile infammation caused by tissue necrosis

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6
Q

Lymphocytic:

A

Chronic inflammation and immunologic stimulation

- viral infection (EBV)

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7
Q

Monocyte:

A

chronic inflammation and infection bacterial endocarditis,

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8
Q

Eosinophilic:

A

allergic disorders such as asthma, hay fever, parasite infection, drug reaction

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9
Q

Basophilic

A

indicative of myeloproliferative neoplasm like chronic myeloid leukemia.

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10
Q

What is reactive neutrophils/

A
  • in severe infections espcecially sepsis show darker and increased granules called toxic granulation.
  • Dohle bodies are pathches o dilated endocardsmic reticum that appear as sky blue cytoplasmic
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11
Q

What about reactive/atypical lymphocytes?

A
  • larger than normal 12 to 16 in diameter
  • Oval, distended or folded nucleus and abundant, sometimes darker cytoplasm with a azurophilic granuels
    MAinly CD8+T cell
    Classically seen in EBV associated infectious mononucleosis
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12
Q

What is leukopenia?

A

Abnormally low white cell count- differntial leukocyte count to check which cells are low
- usually result from reduced numbers of neutrophils

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13
Q

Neutropenia and agranulocytosis:

A

Neutropenia is a reduction in number of neutrophils in the blood
- Agranulocytosis is a marked reduction in enutrophils- serious consequences of making individuals susceptibile to bacterial and fungal infection - most commonly due to drug toxicity

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14
Q

neutropenia can be caused by

A
  1. Inadequate or ineffective granulopoiesis

2. Increased destructionor sequestration of neutrophils in the periphery

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15
Q

Clinical features of neutropenia related to

A

infection and include malaise, chills and fever, often followed by marked weakness and fatigability

  • Agranulocytosis: overwhelming infections and may cause daeth within hours to days
  • Serious infection most likely when the neutrophils falls below 500
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16
Q
  1. Inadequate or ineffective granulopoiesis:
A
  • suppression of HSCs like in aplastic anemia or by tumor infiltration
  • Suppression of committed granulocytic precursors b exposure to certain drugs
  • diseases states associated with ineffective hematopoiesis, such as megaloblastic anemia, and myelodysplastic syndrome, in which defective precursors die in the marrow
  • rare congenital conditions (Kostmann syndrome) inherited defects in specific genes impair granulocytic differentiation
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17
Q

Increased destruction or sequestration of neutrophils in the periphery:

A
  • Immunologically mediated injury to neutrophils- idiopathic, associated with well defined immunologic disorder(SLE) or caused by exposure to drugs
  • ## Splenomegaly- leads to sequestration and destruction of neutrophils in the spleen - modest neutropenia, sometimes associated with anemia and often with thrombocytopeniaIncreased peripheral utilization, which can occur in overwhelming bacterial, fungal, or rickettisal infection.
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18
Q

Introduction to hematoncology:

A
  1. Increased immature cells (no differentiation , maturation)
  2. Increased mature cells-
  3. Defectively matured cells (no maturation)
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19
Q

Difference between leukemia and lymphoma?

A
  1. Leukemia: white blood cells
    - liquid tumors
    - can be myeloid or lymphoid
    - origin ; blood marrow
    - Related to bone marrow
    - Diagnosis: peripheral blood smear, bone marrow aspirate, flow cytometry.
  2. Lymphoma: lymphoid tumor
    : solid tumor
    Only lymphoid
    can be mature or immature
    Any tissue especially lymphoid organs
    related to organ involved like lymphadenopathy
    Diagnosis: biopsy from the tissue organ involved , immunohistochemistry
  • LEukemias can be immature or mature, myeolid or lymphoid
  • lymphomas can be mature or immature, nodal or extranodal ( based on site)
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20
Q

What will happen in bone marrow in case of leukemias?

A
  • Tumor cells start growing in the bone marrow- spill into blood- will raise total counts
  • tumor cells infiltrate and take over bone marrow- no space for normal blood cells to proliferate.
  • *decreased functional cells: decreased RBCs- signs and symptoms of anemia
  • decreased leukocytes- recurrent infections

*Attempt to compensate:
stem cells migrate out of bone marrow to attempt blood cell production in sties like spleen and liver
- extramedullary hematopoiesis : can cause splenomegaly and hepatomegaly and their related symptoms.

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21
Q

Classification of hematological neoplasms:

A
  1. Lymphoid neoplasms: *Immature lymphoid neoplasm-Immature: Acute in onset and presents as leukemia and lymphoma = acute lymphoblastic leukemia/ lymphoma : (ALL)
    * Mature: A special type of lymphoma called Hodgkin lymphoma
    - everything else that is not HL is called non hodgkin lymphoma
  2. Myeloid neoplasm:
    * Immature: acute in onset and presents as leukemia: acute myeloid leukemia
    * Mature: Chronic in onset= chronic myelo-proliferative disorders or myeloproliferative neoplasms
    * Defectively mature: Qualitative defect in cell maturation - disordered growth and maturation = myelo dysplastic syndrome.
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22
Q

Clinical features of hematological neoplasms:Acute leukemias (lymphoid or myeloid)

A
  • Malignant transformation occurs in the early precursors that proliferate uncontrollably without differentiation and maturation
  • Blast cells rapidly proliferate and replace bone marrow- bone marrow failure-presenting in days to weeks- pancytopenia(anemia, infections, bleeding)
  • Hepatosplenomegaly
  • Lymphadenopathy if lymphoid leukemia presents also as a lymphoma
  • Blast cells spill into peripheral blood raising TLC
  • rapidly fatal if not treated
  • Bone marrow transplant - definitive treatment
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23
Q

Clinical features of mature lymphomas:

A
  • Uncontrolled proliferation of neoplastic WBCs that resembles differentiated mature lymphocytes (immature cells =ALL)
  • Not tender lymphadenopathy (if it is tender- usually inflammatory)
  • Can be localized or generalized
  • Non-lymphoid organs involved like skin, central nervous system, GI tract presenting with site specific symptoms of a mass growth
  • signs and symptoms associated with elaboration of substances made by tumor cells like cytokines , autoantibodies etc- night sweats, fever , weight loss etc
    called B symptoms
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24
Q

Diagnosis of hematological neoplasms:

A

CBC and peripheral smear: Alteration in blood cell counts

  • differential leukocyte counts
  • Bone marrow examination: extent of involvement of bone marrow
  • type of tumor cell infiltration
  • Lymph node biopsy or biopsy from the mass: Effacement of normal architecture by tumor lymphoid cells
  • Immunohistochemistry: Done on solid samples (important in lymphoma diagnosis), identify type of tumor cells, identify genetic change
  • Flow cytometry: done on liquid samples(important in leukemia diagnosis) - identify type of tumor cells
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25
Q

What can happen to genes in cancers?

A

*Change at level of gene(DNA)
1. quality: something wrong with the sequence of DNA, mutation, point mutation most common, TESted by PCR.
2, Quantitiy: nothing wrong with sequence, but more copies of the same sequence. Amplification, tested by Fish.

*Change at level of chromosome:
1. Structure: chromosomal rearrangements like transolcation and inversion , tested by karoytping and fish
2. Numbers: Change in ploidy level (not 2n) , trisomy, monosomy, hyperploidy, hypoploidy
,tested by karyotyping.

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26
Q

How do we identify hematologicla cells?

A 
All leucoytes: CD 45
T cells: Cd1,2,3,4,8(especially CD3)
B cells: CD10,19,20,23 (especially CD19)
Myeloid cells: CD13,33, myeloperoxidase
Plasma cells: CD38,138
Stem cells: CD 34
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27
Q

Flow cytometry: every dot is a cell we want to check CD marker expression for

A
  1. These cells are positive for only B (y-axis)
  2. These cells are positive Both A and B
  3. These cells are negative for both A and B
  4. These cells are positive for only A (X-axis)
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28
Q

Normal and reactive lymph node:

A

Capsule, afferent lymphatics,

  • subcapsular sinus (first site of metastasis in a lymph node—— hyperplasia when infiltrated by metastatic tumor cells (sinus histiocytosis)
  • Paracortex: hyperplasia in T cell responses like EBV
  • Cortex: Hyperplasia in B cell rich responses
  • secondary follicle: (after antigen stimulation) b cell rich
  • germinal center
  • mantle zone
  • Medulla: Histiocytes(macrophagesm and plasma cells)
  • Primary follicels: before antigen stimulation.
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29
Q

Overview of lymphoid neoplasms:

A

immature: acute lymphoblastic leukemia/ lymphoma(ALL) : Ball (most common), T-ALL, NK-ALL
predominant symptomology- bone marrow involvement (pancytopenia) +_ Hepatosplenomegaly and lymphadenopathy

  • Mature: Hodgkin lymphoma: Predominant symptomology- non tender localized/generalized
  • Non-Hodgkin lymphoma(NHL): B cell NHL, T cell NHL, cllassifed on the basis of resemblance of neoplastic lymphoid cell to a stage of normal B and T cell development - morphological and genetic resemblance.

When we look at the lymph nodes, and there are proliferation of paracortex ( T cell) - we can suspect viral infection whereas when we look at the lymph nodes, and there are proliferation of cortex(B cell), we can suspect bacterial infection.

*

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30
Q

What are the normal B cell stages?

A

Bone marrow- b-lymphoblast
Peripheral blood- Naive B-cell
Mantle zone of lymphoid follicles (secondary follicle): Mantle cells
Germinal center of lymphoid follicles: Germinal center B cell
Post germinal center of lymphoid follicles: memory B cell, plasma cells

*

31
Q

neoplastic cell stages:

A
  • B- acute lymphoblastic leukemia/ lymphoma(B-ALL)
  • chronic lymphocytic leukemia/small lymphocytic lymphoma
  • Mantle cell lymphoma
  • Burkitt lymphoma, follicular lymphoma, diffuse large B cell lymphoma
  • Marginal zone lymphoma/ MALToma, hairy cell leukemia
  • plasma neoplasms
  • lymphoplasmacytic lymphoma.
32
Q

What is acute lymphoblastic leukemia?

A
  • neoplasms composed of immature B or T cells, which are referred to as lymphoblasts
  • most common cancer of children
    BAll<3 yrs, Tall in adolescents
    PAthogenesis: mutliple choromosomal aberration
    mutation in transcription factor genes
  • B-cell ALL(CD 19+) = more common in children- because the number of normal bone marrow pre B-cell is greatest very early in life
  • more likely to present as leukemia than lymphoma.
  • T cell ALL (CD 3+) = tend to present in adolescent males as thymic lymphomas ( the age when the thymus reaches maximum size)- superior vena cava obstruction may occur
33
Q

Clinical features of ALL?

A
  1. no space for RBC production - anemia- tiredness, pallor, tachycardia if severe
  2. No space for WBC production - Neutropenia(functional cells are less) - fever (most common symptoms), pharyngitis, skin infection
  3. No space for platelet production - thrombocytoenia- mucosal bleeding, petechiae, purpura, epistaxis, menorrhagia
  4. Extramedullary hematopoeisis: hepatosplenomegaly - abdominal distension, dragging sensation in the abdomen.
  5. Involvement of lymph nodes- lymphadenopathy- enlarged non tender lymph nodes
  6. Hypercellular bone marrow- expansion of marrow space- bone pain. (hypercellular bc of filled with tumor cells)
  7. Mediastinal mass(T-ALL) - compression of SVC and airways in mediastinum - dyspnea, facial swelling, cough, chest pain, stridor.
  8. Infiltration of testicular tissue by tumor lymphoblasts: testicular enlargement - unilateral painless enlargement and discomfort
  9. Infiltration of CNS by tumor lymphoblasts: involvement of CNS parenchyma and CSF: headache, vomiting, nerve palsies
34
Q

Investigation of ALL:

A
  1. CBC: raised TLC- lymphoblast increased
    - anemia,thrombocytopenia; reduced mature WBCs
  2. Cytochemistry: myeloperoxidase negative(marker of granulocytes)
    - sudan black B negative (no granules; so no lysosomes- so no lipid membrane to be stained by SBB)
  3. Bone marrow examination - hypercellular marrow (tumor)
    - Lymphoblasts(B or T)
    - Suppression of other cell lines
  4. Biopsy; effacement of normal architecture or infiltration by tumor lymphoid cells
  5. Cytogenetics/ PCR: 90% are abnormal, with the presence of translocation, deleteion and hyperdiploidy
  6. CSF analysis: detection of involvement of brain by tumor lymphoblasts
  7. Flow cytometry: detection of type of lymphoblasts
35
Q

Treatment options of ALL:

A
  • treatment for any acute leukemia is divided into 3 phases: - remission induction: bring down the blast count
  • consolidation therapy: get rid of residual blasts
  • Maintenance therapy

In each phase multi-agent chemotherapy is used (with testicular and CNS prophylaxis- intrathecal)

Overall, 90% achieve complete remission (signs of disease are gone) of which 65% are cured oveall
much higher cure rate in children
Bone marrow transplant is reserved for relapsed cases

  • Lymphoblasts with condensed nuclear chromatin, small nucleoli, and scant agranular cytoplasm (B vs T only possible on flow cytometry/ immunohistochemistry)
36
Q

Prognosis of ALL:

A
  1. Favorable
    - age: 2-10 yrs
    - WBC count: Low WBC
    - Immunophenotype: B cell ALL
    - Cytogenetics: Hyperploidy (more than 46) : Presence of t(12;21)
    - Residual disease after chemotherapy: Undetectable
  2. Unfavorable:
    Younger than 2 yrs or presentation at teens.adult
    - WBC count >100,000 (bc of high tumor burden)
    -T cell ALL
    - Hypo[loidy
    - presence of t(9;22)
    - detectable
37
Q
  1. What is chronic lymphocytic leukemia?
A

Chronic lymphocytic leukemia and small lymphocytic lymphoma differ only in the degree of peripheral blood lymphocytosis

Definition: malignant low grade indolent neoplasm of small mature B cells.
Incidence: most common leukemia in adult
Clinical features: insidious in onset(chronic) - asymptomatic and incidental finding

38
Q

Clinical features of CLL

A
  1. Dysfunction of WBC as and hypogammaglobulinemia- recurrent infections
  2. dysfunctional/ low RBCs: anemia
  3. dysfunctional/ low platelet: mucocutaneous bleeding
  4. Hepatosplenomegaly due to extramedullar hematopoiesis: early satiety, abodominal pain.
  5. Warm autoimmune hemolytic anemia: anemia and jaundice
    Tumor cells involing lymph node: non-tender
39
Q

features of CLL:

A
  • Low power view shows diffuse effacement of nodal architecture
  • high power shows a majority of the tumor cells have the appearance of small, round lymphocytes
  • peripheral smear shows spherocytes and smudge cells(broken fragile tumor cells) along with monotonous tumor cells.
40
Q

Investigation of CLL:

A
  1. CBC: High TLC; differential shows majority of cells are small mature lymphocytes, anemia and thrombocytopenia
  2. Peripheral smear: monotous CLL cells looking like small mature B cells
    , smudge cells, spherocytes (indication of immune hemolytic anemia)
  3. Bone marrow aspirate and biopsy: Lymphoid infiltrates suppressing other cell lines
  4. Lymph node biopsy: effacement of normal architecure by CLL cells spreading diffusely
  5. Flow cytometry/IHC: CD19+,CD20+,CD5+,CD23+
  6. Karyotype: cyogenetic changes like deletion 13q
  7. Coomb’s test: positive for IgG antibodies(warm AIHA)
  • Transformation to a more aggressive neoplasms like a large cell lymphoma- DLBCL - called richter transformation
  • progressive pancytopenia- pneumonia and zoster infections
41
Q

What is Burkitt lymphoma?

A

highly aggressive extranodal maligmant neoplasm of mature B cells resembling germinal center B cells and associated with Epstein Barr virus (EBV)

  • Associated with translocation of the C-MYC gene on chromosome 8
  • Usually translocation occurs with a sequence which increases transcription of MYC leading to overexpression (IHC will be +) like with IgH(heavy chain Ig) on hr.14
  • most common translocation is t(8;14)
42
Q

Endemic / sporadic/ immunodeficiency of burkitt lymphoma:

A
  1. Endemic: EBV= 100%, age = children and young adults, most common= jaws and facial bones, spreads to kidneys, ovaries, adrenals.
  2. Sporadic: EBV= 15-20%m children and young adults, most common= ileocecum and peritoneum, bowel obstruction and gastointestinal bleeding.
  3. immunodeficiency= 25%, no partccular age, most common: CNS, and GIT, symptoms of immunodeficienccy
43
Q

Features of burkitt lymphoma:

A
  1. due to this rapidly proliferation, tumor- starry sky appearance! sky made by rapidly multiplying and dying tumor cells and stars made by pale staining macrophages in between which have come to ingest apoptotic tumor cells!
  2. The tumor cells and their nuclei are faily uniform, giving a monotonous appearance. Note the high level of mitotic activity and prominent nucleoli
    IHC: CD19,20,10,C-MYC
    because of its high proliferation the tumor is aggressive and grows rapidly but also responds well to chemotherapy
44
Q

What is follicular lymphoma?

A
  • malignant indolent neoplasm of mature B cells resembling follicular cells (particularly germinal centers) of lymph nodes and making follicles
  • Pathogenesis: Strongly associated wit chromosomal translocation involving BCL2, hallmark is (14,18)
  • BCL2 = anti-apoptotic
  • seen in up to 90% cases- leads to overexpression of BCL2 (detected on IHC inside the tumor follicles and their germinal centers)

*clinical features: painless, generalized lymphadenopathy
, indolent waxing and waning course- can also undergo Richter transformation

45
Q

features of follicular lymphoma:

A
  • nodular aggregates of lymphoma cells are present throughout lymph node resembling secondary follicles with expanded germinal centers.
  • At high magnification, small lymphoid cells with condensed chromatin and irregular or cleaved nuclear outlines(centrocytes) , are mixed with a population of larger cells with nucleoli (centroblasts)
  • CD19,20,10,BCL2
46
Q

What is diffuse large B cell lymphoma?

A
  • malignant genetically heterogenous neoplasm of mature large B cells growing diffusely
  • most common NHL overall
  • patho: can be primary or secondary (transformation of a low grade small cell lymphoma like)
  • ## molecularly hetergenous-involving BCL2, BCL6, and other genes
47
Q

Clinical features of diffuse large B cell lymphomas?

A
  • rapidly enlarging mass at a nodal(lymph node) or extranodal site
  • can arise virtually anywhere in the body
  • waldeyer ring, the oropharyngeal lymphoid tissue
  • invovlement of the liver and spleen - large destructive masses
  • extranodal site: GIT, skin, brain
48
Q

features of diffuse large B cell lymphomas:

A

CD19,20,10 very heterogenous

tumor cells are thre to fourre size of resting lymphocytes

49
Q

What is marginal zone lymphoma?

A
  • heterogeneous group of mature B cell tumors that arise within lymph nodes, spleen or exta nodal tissue and resemble memory B cell
  • outside of lymph node
    (MALTOMA)- mucosa associated lymph
  • chronically inflamed!!
  • polyclonal chronic inflammation to monoclonal lymphoid proliferation with t(11,18)
  • salivary gland inSjogren diseases, thyroid gland in hashimoto thyroidits, the stomach in helicobacter gastritis.
  • May regressif the inciting agent (H.pylori)
    is eradicated!!
50
Q

Neoplasm , age, sites, special features.

A

ALL: children and adoolsecents , bone marrow, thymus, lymph nodes, CNS and testicular involvement and SVC obstruction, agranular cytoplasm, hepatosplenomegaly, anemia, bleeding, infection

  1. CLL: elderly patients, bone marrow, lymph nodes, , warm autoimmune hemolytic anemia, smugde cells on peripheral smear, can develop to be aggressive DLBCL. diffuse effacement of lymph nodes, positive CD 19,20 , 5,23.
  2. Burkitt lymphoma: children and young adults, Jaw, GIT, CNS, EBV association, endemic, sporadic and immunodeficiency associated, t(8,14) IgH and MYC, Starry sky appearance (macrophages). Jaw tumor/ ileocecal mass.
  3. Follicular lymphoma- adults- lymph nodes- t(14,18)- IgH and BCL2 (anti - apoptosis), indolent ; can transform to DLBCL. closely packed follicles with centrocytes and centroblasts. CD19,20,10,BCL2
  4. DLBCL- adults commonly- nodal and extranodal, molecularly heterogeneous, primary or secondary. diffuse effacement by large cells. CD19,20 , 10
  5. MALToma, adults , extranodal, background of chronicinflammation, t(11,18).positive for CD19,20
  6. HAiry cell leukemia- Adult, anemia, bleeding, infection, hepatosplennogaly, dry tap on bone marrow aspirate, tumor cells with hair like projection , Positive for CD11, 103, Annexin A1.
  7. Plasma Cell neoplasm/multiple myeloma- Adult, anemia, bonepain/ pathological fractures/ infection. renal sufficiency, lytic lesion in bone on X ray, increased plasma cells in bone marrow,
    M band on serum electrophoresis.
    Levels of polyclonal IgG , IgA and lambda light chain are decreased.
    C0 increased serum calcium,
    R- renal insufficiency,
    A- anemia, (due to EPO deficiency leading normochrmic anemia, (kidney effect), tumor cells infiltrating the bone marrow
    B- punched out bone lesion - bone pain and pathological fractures
  8. Classical hodgkin lymphoma- Young adult, painless cervical lymphadenopathy , fever, night sweats, weight loss, neoplastic giant cells with owl’s eye appearancme, positive for CD15,30 but negative for CD 45.
51
Q

Difference between hodgkin lymphoma and non hodgkin lymphoma

A
  1. Hodgkin lymphoma : Nodal»»»». extranodal
    most commonly cervical others: mediastinal, para-aortic
    spread orderly and contiguous(abjacent)
    rare involved (mesenteric and waldeyer ring)
    Neoplastic cells are interspersed between non neoplastic cells
    Young adults and elderly
  2. Non Hodgkin lymphoma
    - can be nodal or extranodal
    - multiple peripheral node involvement more frequent
    - non contiguous
    - commonly involved mesenteric and walderyer ring
    - entre tissue is taken over by neoplastic cells in diffuse or nodular pattern
    - across all ages
52
Q

Hodgkin lymphoma:

A
  • Distinctive features is the presence of neoplastic giant cells called Reed Sternberg cells
  • Owl’s eye appearance
  • release factors that induce the accumulation of reactive lymphocytes, amcrophages,
    RS cell is derived from bcell
  • Curable most cases bc it treated well with radiation therapy and chemotherapy
53
Q

4 subtypes of hodgkin lymphoma:

A
  1. Nodular sclerosis: lacunar type of RS cells and classical RS cells in mixed
  2. mixed cellularity: mixed background of T cells, plasma cells, eosinophils, and macrophages: EBV is postivie, and B symptoms are frequent.
  3. Lymphocyte rich: EBV
  4. Lymphocyte depleted:common in HIB patients.

nodular sclerosis,

54
Q

Nodular lymphocytes predominant hodgkin lymphoma

A

Classical HL: CD 15 , 30 are positive, but negative for CD 45.

Nodular lymphocyte predominant HL: Negative for CD15,30 but positive for CD45

  • no association with EBV
  • excellent prognosis
  • popcorn cell variant
55
Q

T/NK cell lymphomas:

A
  • Mycose fungoides
  • anaplastic large cell lymphoma
  • Adult T cell leukemia
  • peripheral T cell lymphoma,
56
Q

Acute myeloid leukemia:

A

tumor of hematopoietic precursors caused by acuqired oncogenic mutation that impede differentiation leading to the accumulation of immature myeoloid blasts in the marrow.

57
Q

What are the risk factors of AML:

A

Alkylating chemotherpy, raidation, CML progressing to acute leukemias

58
Q

FAB classification:

A 
M0 : acute undifferentiated leukemia
M1: AML without maturation 
M2: AML with maturation
M3: Acute promyelocytic leukemia
M4: Acute myelomonocytic leukemia
M5: Acute monoblastic leukemia
M6: Pure erythroid leukemia
M7: Acute megakaryoblastic leukemia
59
Q

WHO classfication :

A
  1. AML with recurrent genetic abnormalities (including translocation and mutation)
  2. AML, therapy realted
  3. AML with myelodysplasia like features
  4. AML , not otherwise specified
60
Q

Morphological features of AML:

A
  • blasts of myeloid lineas in bone marrow and or peripheral blood more than 20 % - AML
  • Myeloblasts: delicate nuclear chromatin, moderate cytoplasm with myeloperoxidase and Sudan Black B positive granules sometimes fusing to form linear structures called Auer rods
  • Promyelocytes: very granular cytoplasm with many Auer rods and biolobed nuclei.
61
Q

What is promyelocytes:

A

numerous azurophilic granules. Auer rods

62
Q

AML M3 - acute promyelocytic luekemia:

A
  • hypergranular promyelocytes with multiple Auer rods
  • classified as AML with t(15,17) which leads to fusion of PML and RARA genes blocking maturation of promyelocyte to myelocyte.
  • Abnormal granules which are procoagulant risk of disseminated intracellular coagulation.
63
Q

AML M5-

AML M7-

A

Monoblsts infiltrate gums and cause gum hypertrophy.
AML M7: associated with down syndrome.
AML with t (8,21) and inversion 16 - good prognosis
with deletion of chromosome 5q - poor prognosis

64
Q

Clinical features of AML:

A
  1. No space for normal RBC production - anemia
  2. No space for normal WBC production- neutropenia
  3. no space for normal platelet production - thrombocytopenia
  4. Extramedullary hematopoeisis: hepatosplenomegaly
65
Q

Investigation for AML:

A
  1. CBC: Raised RLC- blast of all types are counted by machine under total leucocyte count
    >20% blast in peripheral smear and or blood marrow - AML dianosis
    Schistocytes in case of DIC caused by acute promyelocytic leukemia
  2. Cytochemistry: Myeloperoxidase and Sudan Black B positive in myeloblas.
  3. Bone marrow examination: hypercellular marrow with increased blasts. , suppression of normal cells
  4. Flow cytometry: Best modality for diagnosis - type of AML
  5. PCR and cytogenetics: dianosistic critera based on genetic changes
    - Cytogenetics: diagnosstic criteria based on genetic changes
66
Q

What is chronic myeloid leukemia?

A
  • Characterized by the dysregulated production and uncontrolled proliferation of mature and maturing granulocytes, with normal differentiation.
67
Q

Eitopathogenesis of chronic myeloid leukemia?

A
  • BCR and ABL1 resulting in the BCR- ABL1 fusion gene
  • chromosome 22 and chromosome 9 .
  • fusion protein formed is a tyrosine kinase with constitutive activation.
68
Q

Natural history for CML: patient can present in three phases:

A
  1. Chronic- indolent phase
  2. Accelerated phase : total luekocyte count rises
  3. Blast crisis: number of blasts become more than 20 % and this chronic condition transform into acute leukemia
    1
69
Q

Differential diagnosis of CML:

A

Several infection with leukocytosis can have raised granulocytes like in CML

  • called Leukemoid reaction meaning leukemia like. because it resembles the raised counts of leukemia
  • Can be differentiated by genetic testing, differential leukocyte count and checking quality and quantitiy of NAP. (Neutrophil alkaline phosphatase)
  • NAP score in leukemoid reaction is high but it is low in CML.
70
Q

Clinical features of CML:

A
  • mild to moderate anemia and hypermetabolism due to increased cell turnover lead to faigability , weakness, weight loss, and anorexia
  • splenomegaly
  • fever - non functional WBCs causing recurrent infections
  • Signs of acceleration into blast crisis:
71
Q

pathological findings of CML:

A
  1. Bone marrow: Hypercellular, increased number of all granulocytic cells (mature cells&raquo_space;»> immature cells)
  2. peripheral blood: leukocytosis, neutrophils and myelocytes increased most.
  3. Blast crisis: blast count>20%

CML - peripheral blood smear. Granulocytic forms at various statges of differentiation are present.

72
Q

Cause of polycythemia(increased RBCs)

A
  • Relative polythemia: due to reduced plasma volume
  • Absolute polycythemia (primary): also called polycythemia vera
  • associated with splenomegaly and low EPO(erythropoietin) level.

Secondary: appropriate high EPO levels: lung disease, cyanotic heart disease or living at high altitude
- Inappropriately high EPO levels: EPo- sedreting tumors

73
Q

Difference between polycythemia vera and essential thrombocytosis:

A
  1. Polycythemia vera :
    predominant: erythrocytes, 95% of JAK2 mutation , hemoglobin ?16.5,
    - bone marrow biopsy: hypercellular, erythrocyte hyperplasia
    risk of bleeding and thrombosis: present, hepatic vein thrombosis(Budd chiari syndrome)
    Erythromelalgia: present
    otherfeatures: hypervisocity- heaahcem blurring of vision
    - aquagenic pruitis
    - hyperuricemia and grout.

usmle (7 decks)

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