LEUKOCYTE DISORDERS Flashcards by Janus Palermo

Redistribution of the blood pools causes a short-term increase in the total WBC count and the absolute number of neutrophils in the circulating granulocyte pool; caused by exercise, stress, pain and pregnancy

Shift/physiologic pseudoneutrophilia

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Blood picture mimics chronic myelogenous leukemia; benign, specific response to specific agent or stimulus; WBC count can increase to 50-100x10^9/L; increased LAP score; there is a shift to the left with toxic changes to neutrophils

Neutrophilic Leukemoid Reaction

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Morphologically normal, but functionally abnormal because of enzyme deficiency (NADPH oxidase) that results in an inability to degranulate which causes inhibited bacteriocidal function

Chronic granulomatous disease (CGD)

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what is the treatment for CGD?

granulocyte concentrate

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Both morphologically and functionally abnormal leukocytes; WBCs unable to degranulate and kill invading bacteria; abnormal fusion of primary and secondary neutrophilic granules; leukocytes have gigantic granules that are peroxidase positive

Chediak-Higashi syndrome

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5 or more lobes in the neutrophil; associated with megaloblastic anemia

Hypersegmentation

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Nucleus is hyperclumped and does not mature past the two-fold stage’ nucleus has a dumbbell or peanut-shape; “pince-nez” appearance; morphologically abnormal but functionally normal

Pelger-Huet Anomaly

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What is the difference of Pelger-Huet anomaly and Pseudo Pelger-Huet Anomaly?

In Pseudo PH Anomaly - round nucleus

In Pelger-Huet anomaly - Dumbbell shape nucleus

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Which of the following is not characterized by giant plaetelets?

A. Bernard-Soulier Syndrome
B. Gray Platelet
C. Wiskott-Aldrich Syndrome
D. MHA

C. Wiskott-Aldrich Syndrome

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Large crystalline, Dohle-like inclusions in the cytoplasm of neutrophils on Wright’s stain; gray blue spindle (cigar) shaped; Morphologically abnormal but functionally normal; giant platelets, thrombocytopenia, and clinical bleeding

May-Hegglin anomaly

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Large azurophilic granules appear in the cytoplasm of all or only one cell line; granules contain degraded mucopolysaccharides due to enzyme defect

Alder-Reily Anomaly

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Most common lipid disorder; deficiency in glucocerecrosidase causes glucocerebroside to accumulate in the macrophages of the bone marrow, spleen and liver; Crumpled tissue paper appearance of macrophage

Gaucher disease

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Deficiency in sphingomyelinase causes sphingomyelin to accumulate in macrophages in multiple organs and bone marrow; foamy appearance of macrophages (foam cells)

Niemann-Pick Disease

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What enzyme is deficient in Niemann-Pick disease?

a. Sphingomyelinase
b. glucocerebrosidase
c. Arylsulfatase A
d. Hexosaminidase A

A. Sphingomyelinase

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What enzyme is deficient in Gaucher disease?

a. Sphingomyelinase
b. glucocerebrosidase
c. Arylsulfatase A
d. Hexosaminidase A

B. glucocerebrosidase

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What enzyme is deficient in Fabry’s disease?

a. Alpha galactosidase
b. Cerebroside beta galactosidase
c. Hexosaminidase A and B
d. Hexosaminidase A

A. Alpha galacosidase

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What enzyme is deficient in Tay Sach disease?

a. Alpha galactosidase
b. Cerebroside beta galactosidase
c. Hexosaminidase A and B
d. Hexosaminidase A

D. Hexosaminidase A

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What enzyme is deficient in Krabbe disease?

a. Alpha galactosidase
b. Cerebroside beta galactosidase
c. Hexosaminidase A and B
d. Arylsulfatase A

B. Cerebroside beta galactosidase

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causative agent of IM

Epstein-Barr virus

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what is the target cell of EBV?

B-cells

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Infectious Mononucleosis is also known as “_____”

the kissing disease

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Is IM positive or negative for heterophile antibody test?

yes. positive

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Symptoms similar to IM; most commonly transmitted infection from mother to fetus/ most common congenital infection; negative for heterophile antibody test; no reactive lymphocytes

Cytomegalovirus

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Associated with adenovirus and coxsackie A virus; Contagioyus mostly affecting young children; lymphocytosis with no reactive lymphocytes

Infectious lymphocytosis

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Defines acute leukemia as >30% bone marrow blasts; easier to use and is still widely taught

French-America-British (FAB) Classification

Defines acute leukemia as >20% bone marrow blasts; standard for diagnosis

World Health Organization (WHO) Classification

WHO Classification is based on: (enumerate)

1. Cellular morphology 2. Cytochemical stains 3. Immunologic probes of cell markers (CD markers) 4. Cytogenetics 5. Molecular abnormalities 6. Clinical syndrome

FAB Classification is based on: (enumerate)

1. Cellular morphology 2. Cytochemical stain results

Stains siderotic granules, pappenheimer bodies and hemosiderin

Perl's Prussian blue stain

Stains ALP present in the neutrophil

Leukocyte Alkaline Phsphatase (ALP)

LAP is also known as "_____"

KAPLOW COUNT

LAP score is increased in CML, PNH, Sickle cell anemia, IM, PA and decreased in PV, last trimester of pregnancy, infections with neutrophilia True or False

False LAP is increased in 1. PV 2. Last trimester of pregnancy 3. infections with neutrophilia LAP is decreased in 1. CML 2. PNH 3. Sickle cell anemia 4. IM 5. PA

Stains peroxidase present in granulocytes and monocytes; recommended specimen is fresh blood smear from a capillary puncture

Myeloperoxidase stain (MPO)

MPO is used to differentiate:

Used to differentiate blasts of acute myelogenous leukemias (AMLs) from acute lymphoblastic and monocytic leukemias (ALLs)

These are lysosomes that can be seen in AMLs when stained with MPO

Auer Rods

Stains lipids present in granulocytes and monocytes

Sudan Black B

SBB is used to differentiate _____ and ______ from ______

SBB is used to differentiate Acute myelogenous leukemias and MYELOMONOCYTIC Leukemias from acute lymphocytic leukemia

Stains mucoproteins, glycoproteins, and high molecular weight carbohydrates;

Periodic Acid Schiff stain

PAS stain is used in the diagnosis of?

DiGuglielmo's Syndrome (FAB M6)

In PAS stain, L1 and L2 produces what pattern?

Block pattern

Stains esterases in granulocytes and mast cell granules

Naphthol AS-D chloroacetate esterase

Naphthol AS-D chloroacetate esterase is also known as

Specific esterase

Specific esterase is used to differentiate ______ from _______

Specific esterase is use to differentiate granulocytic cells from monocytic cells

Stains esterases present in the monocytic cells, macrophages, megakaryocytes, and platelets

a-Naphthyl Acetate Esterase

a-naphthyl Butyrate Esterase is also known as

Non-specific esterase

Monocytes stains _____ in non-specific esterase

Red-brown

Marker for HAIRY CELL LEUKEMIA

Tartrate-resistant ACP (TRAP)

Binds with acid mucopolysaccharides in blood cells; useful for the recognition of mast cells and tissue basophils

Toluidine blue

Screening procedure for the detection of chronic granulomatous disease (CGD)

Nitroblue Tetrazolium Neutrophil Reduction Test (NBT test)

Recommended specimen for NBT test

Heparinized whole blood

Positive result in NBT indicates a normal result while the negative result indicates that the patient has CGD True or false

True

A marker for primitive lymphoid cells; used to differentiate ALL from AML

Termimal Deoxyribonucleotidyl Transferase (TdT)

TdT is present in ___% of cases of ALL

TdT is present in 90% cases of ALL

Small lymphoblast, homogenous; Most common ALL in children

FAB L1

large lymphoblast, heterogenous; Most common ALL in Adult

FAB L2

Most common Leukemia in Adult

AML

leukemic phase of BURKITT'S LYMPHOMA

FAB L3

What cells are affected in FAB L3?

B-cell

Blast exhibits myeloid markers; DOES NOT STAIN

FAB M0 - w/o differentiation

What test principle should be used to identify FAB M0 w/o differentiation?

FLOW CYTOMETRY

FAB M0 is detected through CD markers ______

CD 13 CD 33 CD 34 CD 117

>90% of myeloblasts, may have Auer rods

FAB M1 (AML W/O MATURATION)

FAB M1 is positive in stains:

SBB MPO Specific esterase

<90% marrow myeloblasts

FAB M2 (AML WITH MATURATION)

FAB M2 is positive in stains

SBB MPO Specific esterase

>30% marrow PROMYELOCYTES with BUNDLES OF AUER RODS

FAB M3

FAB M3 is also known as:

Hypergranular PROMYELOCYTIC leukemia OR Acute PROMYELOCYTIC leukemia

these are promyelocytes with AUER RODS

Faggot cells

Acute myeloproliferative disorder that is associated with DIC

FAB M3/ Acute promyelocytic leukemia

FAB M3 is positive in stains ____, _____, ______, and negative in _______

positive (+) SBB, MPO, Sp. Esterase negative (-) non. sp. esterase

>30% marrow MYELOBLASTS OF MONOCYTIC ORIGIN

FAB M4

FAB M4 is also known as:

- NAEGELI SYNDROME - Acute MYELOMONOCYTIC Leukemia

FAB M4 is positive in stains:

positive (+) SBB MPO Sp. Esterase Non-Sp. Esterase

FAB M5 is also known as:

SCHILLING LEUKEMIA Acute MONOCYTIC Leukemia

FAB M5 is positive in stains _____ and negative in ______

positive (+) Non-Sp. Esterase negative (-) MPO, SBB, SPE

FAB M6 is also known as:

Di Guglielmo's Syndrome Acute ERYTHROLEUKEMIA

FAB M6 is positive in what stain?

PAS (+) Periodic Acid Schiff stain (+)

FAB M7 is also known as:

Acute MEGAKARYOCYTIC leukemia

Which FAB classification uses anti-vWF Abs and does not use stain to be identified?

FAB M7

The only chronic myeloproliferative disorder that is negative for JAK2 V617F gene

Chronic Myelogenous Leukemia or Chronic Granulomatous Leukemia

In CGL/CML, LAP score is ________

decreased

Philadelphia chromosome is a result of ______ OR ______ gene mutation

Philadelphia chromosome is a result of T (9, 22) and BCR/ABL gene mutation T (9,22) BCR/ABL gene mutation

Plaetelets can reach up to >1000x10^9/L; JAK2V617F gene positive

Essential Thrombocythemia

JAK2V617F gene positive; pancytosis and low EPO

Polycythemia Vera

Increased presence pf smudge cells in the PBS; JAK2V617F gene positive

Chronic Lymphocytic Leukemia

TRAP positive (ACP 5)

Hairy Cell Leukemia

What cell is malignant in HCL?

B-cell

cancer of mature plasma cells; abnormally increased IgG

Multiple Myeloma (MM)

Protein that is present in urine of a patient with MM

Bence Jones Protein

BJP in urine precipitates at _____C and dissolves at _____C

precipitates at 40-60C dissolves at 100C

Lymphoid malignancy characterized with increased IgM; Lymphoplasmacytic infiltration of the BM

Waldenstrom's Macroglobulinemia

The pathognomonic cell of Hodgkin's Lymphoma:

Reed-Sternberg cells

The most common cutaneous lymphoma

Mycosis Fungoides

Mycosis Fungoides is a malignany of

T cells

Cells present in Mycosis Fungoides are called:

Sezary cells - cancerous T cells

lymphoid malignancy caused by HTVL-1

Adult T-cell leukemia

Adult T-cell leukemia is characterized by the presence of:

Flower cells

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS: Stem cell leukemia

lymphoma, undifferentiatedA

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS: Acute lymphoblastic leukemia

lymphoma, poorly differentiated, lymphocytic

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS: Chronic lymphocytic leukemia

lymphoma, well-differentiated, lymphocytic

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS: Monocytic leukemia

Reticulum cell sarcoma

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS: Acute myelogenous leukemia

Chloroma

RELATIONSHIP OF LEUKEMIAS ANBD LYMPHOMAS: Plasma cell leukemia

Myeloma

LEUKOCYTE DISORDERS Flashcards

memorization (103 cards)