Leukocyte Disorder Flashcards
(206 cards)
1
Q
decrease in circulating T cells, poorly functioning B cells, hypogammaglobulinemia, and profound clinical manifestations.
A
SEVERE COMBINED IMMUNE DEFICIENCY
1
Q
Affects both cellular and humoral
A
SEVERE COMBINED IMMUNE DEFICIENCY
2
Q
X-linked SCID
A
GAMMA CHAIN DEFICIENCY
3
Q
most common form of SCID
A
X-linked SCID
4
Q
caused by mutations in the IL2RG gene located at Xq13.1
A
X-linked SCID
5
Q
mutations in the ADA gene located at chromosome 20q13.12
A
AUTOSOMAL RECESSIVE ADENOSINE DEAMINASE (ADA) DEFICIENCY
6
Q
a combined immunodeficiency
A
WISKOTT-ALDRICH SYNDROME
6
Q
X-linked, WAS gene
A
WISKOTT-ALDRICH SYNDROME
7
Q
decreased levels of WASp protein
A
WISKOTT-ALDRICH SYNDROME
8
Q
DiGeorge syndrome, autosomal dominant Opitz GBBB, Sedlackova syndrome, Caylor cardiofacial syndrome, Shprintzen syndrome, and conotruncal anomaly face syndrome
A
22Q11 SYNDROMES
9
Q
X-linked agammaglobulinemia
A
10
Q
Reductions in all serum immunoglobulin isotypes and profoundly decreased or absent B cells
A
BRUTON TYROSINE KINASE DEFICIENCY
11
Q
Autosomal recessive disease of immune dysregulation
A
CHÉDIAK-HIGASHI SYNDROME
12
Q
CHS1 LYST gene on chromosome 1 q42.1-2
A
CHÉDIAK-HIGASHI SYNDROME
13
Q
fused dysfunctional granules
A
CHÉDIAK-HIGASHI SYNDROME
14
Q
partial albinism
A
CHÉDIAK-HIGASHI SYNDROME
15
Q
Defects of mobility
A
LEUKOCYTE ADHESION DISORDERS
16
Q
Decreased ability of neutrophils to undergo a respiratory burst
A
CHRONIC GRANULOMATOUS DISEASE
16
Q
Mutations in genes responsible for reduced form of NADPH
A
CHRONIC GRANULOMATOUS DISEASE
17
Q
Alius-Grignaschi anomaly
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MYELOPEROXIDASE (MPO) DEFICIENCY
18
Q
A deficiency in myeloperoxidase in the primary granules of neutrophils and lysosomes of monocyte
A
MYELOPEROXIDASE (MPO) DEFICIENCY
19
Q
Granulocytes do not respond to chemotactic factors
A
LAZY LEUKOCYTE SYNDROME
20
Q
An uncommon condition where phagocytes with normal random movement but the directional motility of these cells are impaired
A
JOB’S SYNDROME
21
Q
Mutation in the STAT3 gene
A
JOB’S SYNDROME
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warts, hypogammaglobulinemia, infections, and myelokathexis syndrome
WHIM SYNDROME
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Mutations at CXCR4 gene located at 2q22
WHIM SYNDROME
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Decreased nuclear segmentation
PELGER-HUËT ANOMALY
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Distinctive coarse chromatin clumping pattern
PELGER-HUËT ANOMALY
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Mutation in lamin beta-receptor gene
PELGER-HUËT ANOMALY
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Spectacle-like ("pince-nez") morphology
PELGER-HUËT ANOMALY
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Neutrophils with similar morphology to PHA
PSEUDO- OR ACQUIRED PELGER-HUËT ANOMALY
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Neoplastic, infection, drug-induced
PSEUDO- OR ACQUIRED PELGER-HUËT ANOMALY
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> 5 lobes; larger
NEUTROPHIL HYPERSEGMENTATION
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Megaloblastic anemia
NEUTROPHIL HYPERSEGMENTATION
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Granulocytes with large, darkly staining metachromatic cytoplasmic granules
ALDER-REILLY ANOMALY
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Seen in patients with Gargoylism
ALDER-REILLY ANOMALY
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Mucopolysaccharidoses - "Reilly bodies"
ALDER-REILLY ANOMALY
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Mucopolysaccharidoses
"Reilly bodies"
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Thrombocytopenia, giant platelets, large Döhle body-like inclusions
MAY-HEGGLIN ANOMALY
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MYH9 gene on chromosome 22q12-13
MAY-HEGGLIN ANOMALY
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most common of the lysosomal lipid storage diseases
GAUCHER DISEASE
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beta-glucocerebrosidase
GAUCHER DISEASE
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Gaucher disease is a genetic disorder where fat-laden Gaucher cells build up in areas like the spleen, liver & bone marrow.
GAUCHER DISEASE
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accumulation of fat in cellular lysosomes of vital organs
NIEMANN-PICK DISEASE
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acid sphingomyelinase
NIEMANN-PICK DISEASE
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Foam cells and sea-blue histiocyte
NIEMANN-PICK DISEASE
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increased wbc count
Leukocytosis
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decreased wbc count
Leukocytopenia
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leukemoid reaction
reactive leukocytosis
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increased immature neutrophils (bands)
Shift to the left
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Dark, blue-black granules in the cytoplasm of neutrophils
TOXIC GRANULATION
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* Peroxidase positive
TOXIC GRANULATION
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Inflammation, infection, patients
TOXIC GRANULATION
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who have received G-CSF
TOXIC GRANULATION
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RNA
DÖHLE BODIES
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Found in band and segmented neutrophils
DÖHLE BODIES
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Bacterial infections, sepsis, and pregnancy
DÖHLE BODIES
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Phagocytosis
CYTOPLASMIC VACUOLATION
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Bacterial infection, autophagocytosis, secondary to drug ingestion, acute alcoholism, or excess storage of sample before making blood film
CYTOPLASMIC VACUOLATION
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*Found only in females
BARR BODY
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Small, well-defined, round projection of nuclear chromatin
BARR BODY
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Pyknotic nuclei
NUCLEAR AND CYTOPLASMIC CHANGES
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Necrotic nuclei
NUCLEAR AND CYTOPLASMIC CHANGES
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Cytoplasmic swelling
NUCLEAR AND CYTOPLASMIC CHANGES
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any of several atypical lymphocytes seen in infectious mononucleosis.
DOWNEY CELLS
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"Ballerina Skirt Appearance"
DOWNEY CELLS
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mature ones with a kidney-shaped or lobulated nucleus and vacuolated, basophilic, foamy cytoplasm
Type I cells-
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plasmacytoid nuclei with less vacuolated and basophilic cytoplasm
Type II cells
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finer chromatin pattern and 1 or 2 nucleoli.
Type Ill cells
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Viral infections
Binucleated Lymphocytes
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> 5%, lymphocytic leukemia or leukosarcoma
Binucleated Lymphocytes
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nucleus is notched, lobulated, and cloverleaf-like
Rieder Cells
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CLL
Rieder Cells
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Niemann-Pick disease, Tay-Sachs disease,
Hurler syndrome, and Burkitt lymphoma
Vacuolated Lymphocytes
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reaction to viral infections, radiation, and chemotherapy.
Vacuolated Lymphocytes
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leukocytes that have been damaged during preparation of the peripheral blood smear.
Smudge cell
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This usually occurs due to the fragility of the cell.
Smudge cell
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They are usually seen in chronic lymphocytic leukemia (CLL).
Smudge cell
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Either a normal segmented neutrophil or another phagocytic cell with the engulfed homogeneous and swollen nucleus of either a neutrophil or a lymphocyte
LUPUS ERYTHEMATOSUS CELL
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Monocyte that resembles an LE cell but contains a mass with clumped chromatin.
TART CELL
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disease, usually of leukocytes, in the blood and bone marrow. M:E ratio is 10:1
Leukemia
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general term for malignancy that starts in the lymph system, mainly the lymph nodes. Hodgkin and non-Hodgkin
Lymphoma
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a form of cancer of the plasma cells.
Myeloma
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localized tumor masses consisting of myeloblasts may arise in bone or soft tissues
CHLOROMA
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* presence of large quantities of the enzyme MPO produces a green appearance if the tissue is cut
CHLOROMA
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eosinophilic rod-like cytoplasmic inclusions derived from myeloperoxidase-positive primary granules
AUER RODS
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TYPICAL LABORATORY FINDINGS OF AML
* Leukocytosis
* Blastemia
* Leukemic hiatus
* Auer rods in M2, M3, M4
* Thrombocytopenia
* Anemia (normocytic normochromic)
* > 20% blasts in BM
ACUTE MYELOPRO-LIFERATIVE (AML)
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most aggressive of acute leukemia
ACUTE PROMYELOCYTIC LEUKEMIA (FAB M3)
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* severe bleeding tendency and a fatal course, if untreated, of only weeks
ACUTE PROMYELOCYTIC LEUKEMIA (FAB M3)
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* Disseminated intravascular coagulation (DIC) is most often associated
ACUTE PROMYELOCYTIC LEUKEMIA (FAB M3)
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* Referred to as Naegeli Type Monocytic Leukemia
ACUTE MYELOMONOCYTIC LEUKEMIA (FAB M4)
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* AML with t(15;17) in WHO classification
ACUTE PROMYELOCYTIC LEUKEMIA (FAB M3)
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* AML with inv(16) in WHO classification
ACUTE MYELOMONOCYTIC LEUKEMIA (FAB M4)
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* Leukostasis
ACUTE MYELOMONOCYTIC LEUKEMIA (FAB M4)
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* At risk of leukostasis development
ACUTE MYELOMONOCYTIC LEUKEMIA (FAB M4)
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* A pathological finding of slightly dilated, thin-walled vessels filled with leukemic cells.
Leukstasis
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* The brain and lungs are the most commonly involved organs.
Leukstasis
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* Acute erythroid leukemia in WHO classification
ERYTHROLEUKEMIA (FAB M6)
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* Erythremic myelosis or Di Guglielmo syndrome
ERYTHROLEUKEMIA (FAB M6)
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* Proliferation of both immature granulocytic and erythrocytic cell types
ERYTHROLEUKEMIA (FAB M6)
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most common cancer in children (L1)
ACUTE LYMPHOBLASTIC LEUKEMIA
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* Terminal deoxynucleotidyl transferase (TdT) positive
ACUTE LYMPHOBLASTIC LEUKEMIA
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* Common ALL antigen (cALLA) is found on the surface of lymphoblasts in 70% of patients with ALL.
ACUTE LYMPHOBLASTIC LEUKEMIA
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Small homogeneous blasts; mostly in children
L1
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Large heterogeneous blasts; mostly in adults
L2
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"Burkitt type" large basophilic B-cell blasts with vacuoles, t(8;1 4) alteration is common. Frequently a "starry-sky" pattern of admixed histiocytes
L3
90
most common form of leukemia in adults
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
91
*usually a B-cell disorder
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
92
* Commonly exhibit up to 80% or 90% small lymphocytes
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
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* As lymphoma - small lymphocytic lymphoma
CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
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small lymphocytic lymphoma
As lymphoma
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Mature B-cell malignancy
HAIRY CELL LEUKEMIA
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* Pancytopenia
HAIRY CELL LEUKEMIA
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Strong acid phosphatase reaction that is not inhibited by tartaric acid or tartrate-resistant acid phosphatase (TRAP) stain
HAIRY CELL LEUKEMIA
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* a neoplastic proliferation of plasma cells, occurring primarily in the BM
MULTIPLE MYELOMA
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*normochromic, normocytic anemia
MULTIPLE MYELOMA
100
* Produce multiple tumors, which appear on a radiograph as multiple punched-out osteoporotic lesions
MULTIPLE MYELOMA
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* marked degree of rouleaux formation
MULTIPLE MYELOMA
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* Monoclonal gammopathy, Hyperviscosity of the blood, Bence-Jones protein in the urine
MULTIPLE MYELOMA
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B-cell neoplasm characterized by lymphoplasmoproliferative disorder with infiltration of the bone marrow and a monoclonal immunoglobulin M (IgM) protein.
WALDENSTRÖM MACROGLOBULINEMIA
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* a malignancy of the lymphoplasmacytoid cells, which manufacture IgM.
WALDENSTRÖM MACROGLOBULINEMIA
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*Monoclonal gammopathy, Hyperviscosity of the blood, Bence-Jones protein in the urine
WALDENSTRÖM MACROGLOBULINEMIA
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divided into Hodgkin and non-Hodgkin types
LYMPHOMA
105
* Hodgkin type
‣ characterized by the presence of Reed-Sternberg cells
LYMPHOMA
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‣ characterized by the presence of Reed-Sternberg cells
Hodgkin type
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A large binucleated, multinucleated, or mononuclear (Hodgkin) cell with each nucleus bearing a very large inclusion-like nucleolus
Reed-Sternberg cells
108
a large multinucleated cell resembling an "owl's eye.
Reed-Sternberg cells
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leukemic phase of cutaneous T-cell lymphoma, mycosis fungoides
SÉZARY SYNDROME
110
Rare atypical mononuclear cells with cerebriform nuclei
Sezary cells
111
an MPN that originates in an abnormal pluripotent bone marrow stem cell and is consistently associated with the BCR-ABL 1 fusion gene located in the Philadelphia chromosome
Chronic Myelogenous Leukemia (CML)
111
1. Chronic Myelogenous Leukemia
2. Polycythemia vera
3. Primary myelofibrosis (also known as agnogenic myeloid metaplasia or myelofibrosis with myeloid metaplasia)
4. Essential thrombocytosis or essential thrombocythemia
MYELOPROLIFERATIVE NEOPLASMS
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reciprocal translocation of DNA between chromosomes 9 and 22 (t9q:22q)
Philadelphia chromosome
113
main portion of the long arm of chromosome 22 is deleted and translocated to the distal end of the long arm of chromosome 9.
Philadelphia chromosome
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(t9q:22q)
Philadelphia chromosome
115
Lap score <10
Chronic Myelogenous Leukemia (CML)
116
An absolute increase in red cell mass, leukocytosis, and thrombocytosis.
POLYCYTHEMIA VERA
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* Pancytosis in peripheral blood
POLYCYTHEMIA VERA
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* Panhyperplasia of the bone marrow
POLYCYTHEMIA VERA
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Phlebotomy → IDA
POLYCYTHEMIA VERA
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PV characterized by mutation of
Janus 2 kinase, JAK2 V617F
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* Serum and urine erythropoietin are decreased
POLYCYTHEMIA VERA
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* Systemic bone marrow fibrosis and extramedullary hematopoiesis
PRIMARY MYELOFIBROSIS
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* is caused by infiltrative disorders, including malignancies and infections, or exposure to chemical toxins or irradiation.
Secondary myelofibrosis
122
Leukoerythroblastic picture of teardrop-shaped erythrocytes, nucleated erythrocytes, and immature myeloid cells
PRIMARY MYELOFIBROSIS
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disorders in which apoptosis predominates, hematopoiesis is ineffective, and cytopenias occur
MYELODYSPLASTIC SYNDROMES
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a clonal myeloproliferative disorder primarily affecting the megakaryocytic lineage with the principal manifestation of sustained thrombocytosis
ESSENTIAL THROMBOCYTHEMIA
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Chronic myelomonocytic leukemia (CMML),
MYELODYSPLASTIC SYNDROMES
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Atypical chronic myeloid leukemia (aCML),
MYELODYSPLASTIC SYNDROMES
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BCR-ABLi negative, juvenile chronic myelomonocytic leukemia (JCMML)
MYELODYSPLASTIC SYNDROMES
128
Myelodysplastic/ myeloproliferative neoplasm, unclassifiable
MYELODYSPLASTIC SYNDROMES
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dysmyelopoietic syndromes or preleukemias
MYELODYSPLASTIC SYNDROMES
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Dyserythropoiesis
MYELODYSPLASTIC SYNDROMES
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Dysgranulopoiesis
-pseudo-Pelger-Huët
MYELODYSPLASTIC SYNDROMES
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Dysgranulopoiesis
MYELODYSPLASTIC SYNDROMES
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Dysmegakaryocytopoiesis
-giant hypogranular platelets
MYELODYSPLASTIC SYNDROMES
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-giant hypogranular platelets
Dysmegakaryocytopoiesis
135
Purpose: To differentiate a myelogenous or monocytic leukemia from acute lymphocytic leukemia
MYELOPEROXIDASE
136
is present in the primary azurophilic granules of neutrophil, eosinophil and monocyte & activity increased with maturation, no activity is found in red cells or lymphocytes.
Peroxidase
137
The red granular staining peroxidase activity.
MYELOPEROXIDASE STAIN, BONE MARROW ASPIRATE
138
is fat soluble, then it stains fat particles (Steroles, phospholipids and neutral fats) which present in the primary and secondary granules of myelocytic and monocytic cells.
Sudan black B
138
To distinguish acute myelogenous and monocytic leukemia from lymphocytic leukemia.
SUDAN BLACK B
139
stain in a patient with AML
Positive sudan black B (SBB)
140
show coarse staining granules with a faint staining pattern for myeloblast and increase staining with maturation.
Myelogenous cells
141
show finely scattered granules.
Monocytic cells
142
Differentiate myelocytic and monocytic leukemia.
NON SPECIFIC ESTERASE: {WITH FLUORIDE INHIBITION}
143
When naphthol AS-D chloroacetate is used as a substrate, the reaction is positive in the granulocytic cells and negative to weak in the monocytic cells
SPECIFIC ESTERASE OR CHLOROACETATE
144
stain in a patient with AML,M2. Note the bright red staining indicating that these two blasts are of myeloid origin.
Naphthol (AS-D) Chloroacetate Esterase
145
the stain indicates the presence of mucoproteins, glycoproteins and high molecular weight carbohydrates in blood cells.
PERIODIC ACID - SCHIFF [PAS] REACTION
146
M6 (Diguglielmo syndrome)
PERIODIC ACID - SCHIFF [PAS] REACTION
147
subtypes of AML
PERIODIC ACID - SCHIFF [PAS] REACTION
148
Diagnosis of some acute lymphocytic leukemia
PERIODIC ACID - SCHIFF [PAS] REACTION
149
(+ve) brick - red staining which found in:
*Megakaryocyte and platelets, Histiocyte, Macrophage, Monocyte & Lymphoblast of ALL
NON SPECIFIC ESTERASE: {WITH FLUORIDE INHIBITION}
150
The addition of sodium fluoride to the incubation solution inhibits the staining reaction in monocytes but not in granulocytes.
NON SPECIFIC ESTERASE: {WITH FLUORIDE INHIBITION}
151
Neutrophils are positive at most stages of development, but most strongly in the mature stage.
PERIODIC ACID - SCHIFF [PAS] REACTION
152
Myeloblasts are usually weakly positive or negative.
PERIODIC ACID - SCHIFF [PAS] REACTION
152
Eosinophils are positive at all stages of development
PERIODIC ACID - SCHIFF [PAS] REACTION
152
Lymphocytes may contain a few positive granules, but in lymphocytic leukemias the malignant lymphocytes may have an increased number of PAS-positive granules in a focal or block-like positivity.
PERIODIC ACID - SCHIFF [PAS] REACTION
152
Monocytes are weakly positive in the form of fine granules.
PERIODIC ACID - SCHIFF [PAS] REACTION
153
NRBCs are negative, but stain positive in the abnormal erythroid precursors of erythroleukemia.
PERIODIC ACID - SCHIFF [PAS] REACTION
154
Megakaryocytes stain positive.
PERIODIC ACID - SCHIFF [PAS] REACTION
155
Distinguishing the cells of leukemoid reactions with increase activity from these of (CML) with decreased activity.
LEUKOCYTE ALKALINE PHOSPHATASE (LAP)
156
is present in varying degrees in the neutrophil and band form of the granulocytes / some times in B lymphocytes
Alkaline phosphatase Activity
157
WILL NOT AFFECT LAP RESULT:
* Untreated hemolytic anemia
* Lymphosarcoma
* Viral hepatitis
* Secondary polycythemia
158
Siderotic granules are found in the cytoplasm of developing cells in [BM] in the form of Ferric [Fe+ 3]
PRUSSIAN BLUE REACTION
159
Perls' reagent is formed of (Potassium Ferricyanide + HCI)
PRUSSIAN BLUE REACTION
160
Siderotic granules are found in nRBCs, some reticulocytes
PRUSSIAN BLUE REACTION
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163
164
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166
167
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169
170
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172
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175
204
X-linked SCID caused by mutations in the IL2RG gene located at
Xq13.1
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