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1. hematology > White blood cell disorders > Flashcards

White blood cell disorders Flashcards

1
Q

benign leukocyte disorders

A

benign leukocyte disorders are non- neoplastic conditions affecting WBCs
They can be qualitiative or quantitative disorders
Quantitive (numerical) leukocytes disorders:
increased WBC numbers (cytoses)
Decreased WBC numbers (cytopenia)

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2
Q

quantitative leukocyte disorders that dont fit in the cytopenia or cytoses categories

A

leukemoid reaction

Leukoerythroblastic reaction

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3
Q

leukemoid reaction

A

a benign, exaggerated response to infection it is NOT leukemia >50,000 uL
Neutrophils, lymphocytes, or eosinphils
Perforating appendicits- neutrophilic leukemoid reaction
Whooping cough (bordetella pertussis) - Lymphocytic leukemoid reaction
Nematode infestation (cutaneous larva migrans)- eosinophilic leukemoid reaction

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4
Q

leukoerythroblastic reaction

A
  • Presence of immature bone marrow cells in the peripheral blood
  • bone marrow infiltrative process such as bone marrow fibrosis or metastatic cancer leads to immature WBCs (blasts) or RBCs (nucleated RBCs) in peripheral blood
  • Neoplastic conditions that are accompanied by leukoerythroblastic reaction
  • Severe bone marrow stress due to benign conditions (sepsis, growth factor admin)
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5
Q

Neutrophilia

A

Absolute neutrophil >7000 uL
Infection (acute appendicitis)
Sterile inflammation with Necrosis (acute MI)
Drugs (G- CSF, Steroids catecholamines, lithium)
In an infection, Nuetrophils increase in number, but also have prominent blue primary granules (instead of secondary orange granules)– TOXIC CHANGE

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6
Q

Neutropenia

A

Absolute neutrophil <1500/uL

Apalastic anemia, immune destruction, septic shock, chemotherapy

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7
Q

Eosinophilia

A 
Absolute eosinophil count>700/uL
Type 1 HS
Invasive helminths
Hypocortisolism (addisons)
Neoplasms (hodgkins lymphoma)
Morphologically the same as normal eosinophils
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8
Q

Basophilia

A

Absolute basophil>200uL
Allergic and hypersensitivity disorders
Infections
Chronic kidney disease

CHRONIC MYELOID LEUKEMIA

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9
Q

Myeloproliferative neoplasms (MPNs) and Myelodysplastic syndromes (MDSs)

A

Members of the larger category of Myeloid neoplasms
Acute myeloid leukemia

a set of neoplastic stem cell disorders that involve one or more cell lineages

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10
Q

Myeloproliferative neoplasms (MPNs) (definitions, classification)

A

Clonal hematopoietic stem cell disorders characterized by proliferation of one or more of the myeloid lineages (granulocytic, erythroid, or megakaryocytic)

Usually all cell lineages are increased but one is the most
CML, PV, PMF, and ET

More common in adults, hypercellular bone marrow with effective hematopoiesis (incresed numbers of cells in the peripheral blood)
Usually splenomegaly and/or hepatomegaly, potential for disease progression to bone marrow fibrosis or acute leukemia

almost all MPNs have have INCREASED TYROSINE KINASE activity

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11
Q

MPN vs MDS

A

Both MPN and MDS are neoplastic condtions (pre acute leukemia states),

MPNs are hyper cellular bone marrow with INCREASED CELL PROLIFERATION, cytosis

MDS is hypercellular bone marrow with INNEFFECTIVE HEMATOPOIESIS - DECREASED peripheral blood, cytopenias- MDS promotes cell proliferation and cell death, but cell death out ways it

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12
Q

Chronic myeloid leukemia (CML)

A

BCR- ABL 1 fusion gene positive–> produces tyrosine kinase
neoplastic proliferation of mature myeloid cells GRANULOCYTES and their precursors also characteristically BASOPHILS!!!!!! extremely high leukocytosis (>100k/uL( with neutrophila and immature myeloid cells, rare myeloblasts, BASOPHILIA, thrombocytisis

hypercellular BM and granulocytic hyperplasia

The BCR-ABL1 fusion gene is driven by the PHILIDELPHIA chromosome t (9,22) leads to increased tyrosine kinase (seen via FISH, RT PCR)

1st line therapy is IMATINIB tyrosine kinase inhibitor, Splenomegaly is common, enlarging spleen is indicative of progression to accelerated phase of disease, transformation to acute leukemia usually shortly thereafter (to AML2/3 or ALL 1/3) mutation is in the pluripotent stem cell

CML is different from a leukemoid reaction–negative leukocyte alkaline phosphatase, Increased BASOPHILS, philidelphia chromosome

Chronic phase (3 years), accelerated phase (1 year), blast phase (acute leukemia >20% blasts in PB or BM)

NO cure, eccept allogeneic stem cell transplant

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13
Q

Polycythemia vera (PV)

A

A MPN of mature myeloid cells, especially RBCs
granulocytes and platelets are also increased
JAK 2 kinase mutation–> increased tyrosine kinase
Clinical symtoms due to hyperviscosity of blood (blurry vision and headache, increased risk of venous thrombosis, flushed face due to congestion, itching after bathing), splenomegaly, goutm histamine release

Treatment is phlebotomy, and hydroxyurea
Death in one year
How PV is different from reactive polycythemia (in PV EPO is decreased and SaO2 is normal, reactive due to high altitude or lung disease SaO2 is low and EPO is increased., Reactive polycytemia is due to ectopic EPO is made peripherally and is hifh, and SaO2 is normal

To say someone has PV they need to have JAK2 mutation and hypercellular BM with erythroid hyperplasia

PV usually have a long clinical course (>10 year) most pts die of thrombosis or hemorrhage, 15-20% pts have a spent phase (Primary myelofibrosis), 2-3 % of patients may develop a MDS or AML

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14
Q

Primary myelofibrosis (PMF)

A

an MPN of mature myeloid cells especially megakaryocytes, produces excessive PDGF, causing bone marrow fibrosis and extramedullary hematopoiesis(EMH) in the spleen, liver and lymph nodes. Pts get splenomegaly, causing portal HTN
Early prefibrotic PMF show peripheral blood leukocytosis, typical fibrotic PMF shows normal anemia, TEAR DROP RBCs (theyre struggling to get out of the fibrotic material)

JAK 2 mutation is found in 50% of cases (3-7 year survival for fibrotic and >10 for prefibrotic), pts may devleop AML

Usually EPO binds JAK and then JAK tells the cell to start proliferating, in fibrosis, JAK is always phosphorylated–> excessive proliferation

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15
Q

Essential Thrombocythemia (ET)

A

An MPN characterized by a proliferation of megakaryocytes– elevated platelet count in peripheral blood, with an atypical morphology, RBC

Associated with JAK2 mutations, increased risk of bleeding or thrombosis, rarely progresses to marrow fibrosis or Acute leukemia, no real risk for gout/hyperuricemia

BM is hypercellular w/abnormal megakaryocytes

survival is relatively long, treatment is alkylating agents to lower the platelet count

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16
Q

Myelodysplastic syndromes (MDS)

A

MDSs are clonal hematopoietic stem cell disorders with cytopenia, dysplasia in one or more myeloid cell lineages. Ineffective hematopoiesis and increased risk of developing AML

Enhanced degree of apoptosis–> cytopenia
MYELOBLASTS ARE ALWAYS <20% in PB and BM cut off for AML

AML (20 % or more in PB and BM), MDS (less than 20% myeloblasts)

MDS: cytopenias, dysplastic features, a hypercellular BM, ring sideroblasts, chromosomal abnormalities
hypogranular and/or hypolobulated PMNs, large, hypogranular platelets, multinucleated red cell precursors, small hypolobulated megakaryocytes in BM aspirate, ring sideroblasts (occur due to abnormal iron accumulatin in impaired mitochondria)

17
Q

MDS clinical and treatment

A

MDS is more frequent in elderly individuals, weakness, infection or hemorrhage
Survival 1-3 years, progression to AML (30%)
hard to cure, managed supportively with blood products, Antibiotics, growth factors, hypomethylating agents (decitabine azacytidine), these drugs reduce bone marrow cellularity, the precentage of blasts, and improve cytopenias

Allogenic stem cell transplant only cure

18
Q

Acute myeloid leukemia general

A

acute leukemias are neoplastic proliferations of immature cells blasts, recapitulating progenitor cells of the hematopoietic system
2 different types of acute leukemias:
Myeloblastic/myeloid vs lymphoblastic

From a clinical standpoint, acute leukemias are proliferations of immature cells for weeks to months. Chronic leukemias are proliferations of mature cells and have a history of months to years

19
Q

Acute myeloid leukemia (AML) vs Acute lymphoblastic leukemia

A

ALL is more common in children, seen in adults too
AML is more in adults (Mature= aMl)
AML and ALL therapy is very different and prognosis is different

20
Q

Cytochemistry differences between AML vs ALL

A

can tell appart the presence of intra cellular enzymes, different colors)

AML has myeloperoxidase (MPO generates a black intracellular product) and NSE (non specific esterase, a brick red intracellular product)

21
Q

immunophenotyping of AML vs ALL

A

can also identify subtypes of AML
immunohistochemistry uses antibodies,flow cytometry uses tagged Ab that recognize Ag expressed by cells in cell suspension

for AML, markers of immaturity CD34 and CD117

22
Q

Acute myeloid leukemia (AML)

A

Generally a poor outcome (survival of 25%)
AUER RODS, AUER RODS, AUER RODS, AUER RODS!!!
Usually has cytopemia (weakness fatigue and petechiae, infections)
Organomegaly, lymphadenopathy and infiltration
Coagulopathy

MAIN diagnostic factor >20% of myeloid basts! in the blood or marrow
bone marrow is hypercellular

peripheral blood can be severe leukopenia to marked leukocytosis, anemia and thrombocytopenia
AML with recurrent cytogenetic abnormalities(favorable prgnosis), AML with myelodysplasia (poor prognosis), therapy myeloid neoplasms, Myeloid with down syndrome

23
Q

AML with recurrent cytogenetic abnormalities

A 
t(8,21)- RUNX1
inv (16)- CBF
t(15, 17)-PML
t(9,11)- KMT2A
t (6,9)- DEK

t(8,21), inv 16, t(15,17)- good prognosis
t 9/11- intermediate
t 69 - poor prognosis

24
Q

AML with t(15,17)

A 
AML with t(15,17) or acute promyelocytic leukemia (APL)
Atypical promyelocytes (hypergranular),reniform/ bilobed nuclei and occasional cells show multiple prominent AUER RODS

typical hypergranular cases of APL usually show leukopenia, although microgranular variant often manifests leukocytosis
AML is important because it frequently manifests disseminated intravascular coagulation

responds to ALL TRANS RETINOIC ACID (ATRA) which effectively makes it a favorable AML

t(15,17) PML RARA- retinoic acid, important for retinoic acid is myeloiod maturation. RAR disruption blocks maturation and promyelocytes accumulate, ATRA overcomes it and matures the cells, and corrects coagulopathy

25
Q

Langerhans cell histiocytosis

A

Proliferation of dendritic cells or macrophages
Langerhans cells are immature Dendritic cells in the skin
Found in many organs, capture Ags and display rhm to T cells

Proliferating langerhans cells express MHC II, CD1 a and langerin (a transmembrane birbeck granules) – give a tennis racket appearance

Abundant vacuolated cytoplasm and vesicular nuclei- histiocytes
Multisystem Langerhans cell histiocytosis (Letterr Siwe disease), kids under 2, multifocal cutaneous lesions, hepatosplenomegaly, lymphadenopathy, pulmonary lesions and destructive bone lesions, pancytopenia

Unisystem langerhans cell histiocytosis (eosinophilic granuloma)- benign proliferations of langerhans in bone
Hand shuller christian triad- BRAF

26
Q

Hemophagocytic lymphohistiocytosis/ hemophagocytic syndrome

A

HLH is a potentially fatal hyperinflammatory condition

causes perforin and granzymes, primary HLH– familial HLH
Infectious, rheumatologic, malignant, metabolic conditions

Immune sysntem kills organs

A secondary disorder

27
Q

AML epidemiology

A

Rarely inherited, 30% of adult leukemias, etiology 10% therapy related

Favorable: Inv 16, t (16,16), t(8,21), t(16,17) (multiples of 8 or 7)
Intermediate (normal, 9,11)

Poor risk (11q23, non 9 11, 3 ,3 6,9 , 9 22)

28
Q

AML therapy

A

Induction (7+3 with Ara- C and daunorubicin Midostaurin and Gemtuzumab)

Consolidation (Chemotherapy, stem c3ell transplant)

Target Threapy : IDH 1 mutation: Ivosidenb,
IDH2: Enasidenib,
FLT3:Giltretinib

29
Q

CML

A

Clonal myeloproliferative disorder of puripotent stem cells, Increased proliferation, decreased apoptosis
Philidelphia chromosome, BCR?ABL

7% to 15% of adult leukemias

Incidence 1.5/10x5 , prevalence 5/20^5

Irradiation ais a cause

USE A TYROSINE KINASE INHIBTOR (imatinib, dastinib, bosutinib, ponatinib)

30
Q

MDS epidemiology

A

Risk increases with age, M>F, Whites> colored, 3 yr survival 35%

31
Q

MDS Therapy

A

Stem cell transplant, Supportive care, hypomethylating agents, lenalidomide

Hypermethylation causes gene silencing, So then a hypomethylating agent would allow for the apoptotic gene to be upregulated

1. hematology (8 decks)

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