LEUKOCYTE DISORDERS Flashcards
memorization
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
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
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)
what is the treatment for CGD?
granulocyte concentrate
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
5 or more lobes in the neutrophil; associated with megaloblastic anemia
Hypersegmentation
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
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
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
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
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
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
Deficiency in sphingomyelinase causes sphingomyelin to accumulate in macrophages in multiple organs and bone marrow; foamy appearance of macrophages (foam cells)
Niemann-Pick Disease
What enzyme is deficient in Niemann-Pick disease?
a. Sphingomyelinase
b. glucocerebrosidase
c. Arylsulfatase A
d. Hexosaminidase A
A. Sphingomyelinase
What enzyme is deficient in Gaucher disease?
a. Sphingomyelinase
b. glucocerebrosidase
c. Arylsulfatase A
d. Hexosaminidase A
B. glucocerebrosidase
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
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
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
causative agent of IM
Epstein-Barr virus
what is the target cell of EBV?
B-cells
Infectious Mononucleosis is also known as “_____”
the kissing disease
Is IM positive or negative for heterophile antibody test?
yes. positive
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
Associated with adenovirus and coxsackie A virus; Contagioyus mostly affecting young children; lymphocytosis with no reactive lymphocytes
Infectious lymphocytosis
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)
- Cellular morphology
- Cytochemical stains
- Immunologic probes of cell markers (CD markers)
- Cytogenetics
- Molecular abnormalities
- Clinical syndrome
FAB Classification is based on: (enumerate)
- Cellular morphology
- 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
