FINALS: Nonmalignant Granulocytic Disorders Flashcards
Redistribution of the blood pools cause a short-term increased in the total WBC count and in the absolute number of neutrophils in the circulating granulocyte pool.
Shift/physiologic/pseudoneutrophilia
Caused by exercise, stress, pain, pregnancy
Shift/physiologic/pseudoneutrophilia
It is not a response to tissue damage. The total blood granulocyte pool in the body has not changed.
Shift/physiologic/pseudoneutrophilia
The bone marrow has not released immature neutrophils. There are no toxic changes. And there is ______ to the left.
no shift
Neutrophils leave the circulating pool, enter the marginating pool, and then move to the tissues in response to tissue damage
Pathologic neutrophilia
In pathologic neutrophilia, bone marrow reserves are _____ to replenish the circulating pool and ______ production of neutrophils to replenish reserves.
released
increases
Occurs in response to bacterial and the other infections, tissue destruction, drugs or toxins, growth factor, etc.
Pathologic neutrophilia
Blood picture mimics that seen in chronic myelogenous leukemia.
Neutrophilic leukemoid reaction (NLR)
Benign, extreme response to a specific agent of stimulus
Neutrophilic leukemoid reaction (NLR)
In neutrophilic leukemoid reaction (NLR), the WBC count can _____ to between 50.0 and 100.0×〖10〗^9/L, and there is a ______ with ______ changes to the neutrophils.
increase
shift to the left
toxic
Characterised by having the presence of immature leukocyte and immature (nucleated) erythrocytes in the blood
Leukoerythroblastic reaction
Occurs in marrow replacement disorders, such as myelofibrosis
Leukoerythroblastic reaction
Decrease in absolute number of neutrophils
Neutropenia
Due to bone marrow production defects chronic or severe infection depletes available neutrophil reserves. Use exceed bone marrow production
Neutropenia
____ causes neutrophils to be removed from circulation
Hypersplenism
Neutropenia can be cause by one of the ff:
bone marrow injury (aplastic anemia),
bone marrow infiltration (leukemia, myelodyplastic syndromes or mestatistic cancer),
bone marrow suppression by chemicals or drugs (chemotheraphy)
Neutropenia can be cause by DNA synthesis defects due to
vitamin B12 or folate deficiency
Increase in the absolute number of eosinophils
Eosinophilia
Associated with parasitic infections, allergic reactions, chronic inflammation
Eosinophilia
Associated with chronic myelogenous leukemia, including early maturation stages, Hodgkin disease, tumors
Eosinophilia
Decrease in the absolute number of eosinophils
Eosinopenia
Seen in acute inflammation and inflammatory reactions that cause release of glucorticosteroids epinephrine
Eosinopenia
Increase in the absolute number of basophils
Basophilia
Associated with Type I hypersensitivity reactions
Basophilia
Associated with chronic myelogenous leukemia, including early maturation stages, polycythemia vera
Basophilia
Associated with relative transient basophilia can be seen in the patient on hematopoietic growth factors.
Basophilia
Decrease in the absolute number of basophils associated with inflammatory states and following immunologic reactions
Basopenia
Chronic granulomatous disease (CGD) : Nitroblue Tetrazolium Test (NBT) is _________ inheritance with the ratio of affected males to females being 6:1
both sex-linked and autosomal recessive
In Chronic granulomatous disease (CGD) : Nitroblue Tetrazolium Test (NBT), granulocytes are morphologically _______, but functionally ______ because of enzyme deficiency that results in an inability to degranulate, with causes inhited bactericidal function.
normal
abnormal
Characterised as an autosomal recessive disorder that causes large, gray-green, peroxidase positive granules in the cytoplasm of leukocytes; abnormal fusion of primary and secondary neutrophilic granules
Chédiak-Higashi syndrome
In Chédiak-Higashi syndrome, cells are morphologically ______ and functionally _______ leukocyte; WBCs unable to degranulate and kill invading bacteria
abnormal
abnormal
Patients will present with photophobia and skin hypopigmentation (ALBINISM)
Chédiak-Higashi syndrome
Characterised by normal random activity but characterized by abnormal chemotactic/directional activity
Job’s syndrome
Characterised by abnormal random activity and abnormal chemotactic activity
Lazy Leukocyte Syndrome
Hereditary hypersegmentation of neutrophil – autosomal dominant trait: _______
UNDRITZ Anomaly
Acquired hypersegmentation of neutrophil is associated with_______ anemia due to vitamin B_12 or folic acid deficiencies
megaloblastic
_______ refers to a tendency in neutrophils to have 1 or 2 lobes; may indicate an anomaly or a shift to the left.
Hyposegmentation
An autosomal dominant inheritance that is characterised by having a nucleus that is hyperclumped, and it does not mature past the two-lobed stage.
Pelger –Huët anomaly
Shape of neutrophils’ nucleus in Pelger –Huët anomaly
Nucleus dumbbell- or peanut shaped;
Shape of neutrophils’ nucleus in Pelger –Huët anomaly is referred to as
“pince-nez”
Neutrophils affected by Pelger –Huët anomaly is morphological ____ and functionally _____
abnormal
normal
Pelger –Huët anomaly must be differentiated from a shift to the left associated with an infection (toxic changes); infection requires treatment but Pelger-Huët anomaly (does not need or needs treatment????)
(no toxic changes) does not.
Acquired abnormality associated with myeloproliferative disorders and myelodysplastic syndromes; can also be drug induced
Pseudo Pelger-Huët
Appearance of Pseudo Pelger-Huët nucleus is usually
round instead of a dumbbell shape that is seen in the anomaly.
Pseudo Pelger-Huët is frequently accompanied by____-
hypogranulation
An autosomal dominant inheritance characterised by having large, crystalline, Döhle-like inclusions in the cytoplasm of neutrophils on Wright’s stain;
May-Hegglin anomaly
Appearance of the inclusions in May-Hegglin anomaly with Wright’s stain
gray-blue and spindle (cigar) shaped
In May-Hegglin anomaly, cells are morphologically ______, functionally ______
abnormal
normal
Giant platelets, thrombocytopenia, and clinical bleeding are also associated with this anomaly.
May-Hegglin anomaly
An autosomal recessive inheritance characterised as having large azurophilic granules appear in cytoplasm of all or only one cell line. Granules contain degraded mucopolysaccharides due to an enzyme defect
Alder-Reilly anomaly
In Alder-Reilly anomaly, cells are morphologically_____, functionally_____
abnormal
normal
Increase in the absolute number of monocyte associated with recovery stage from acute bacterial infections and recovery following marrow suppression by drugs
Monocytosis
Increase in the absolute number of monocyte associated with tuberculosis, syphilis, subacute bacterial endocarditis
Monocytosis
Increase in the absolute number of monocyte associated with autoimmune disorders (systemic lupus erythematosus, rheumatoid arthritis)
Monocytosis
Lipid storage disorders
Gaucher disease
The most common lipid storage disorder and has an autosomal recessive inheritance pattern.
Gaucher disease
Gaucher disease has a deficiency in _______ causes glucocerebroside to accumulate in macrophages of the bone marrow, spleen and liver, with Gaucher cells (chicken scratch appearance) more commonly seen in the bone marrow.
glucocerebrosidase/B-glucosidase
Niemann-Pick disease is an autosomal recessive inheritance pattern. A deficiency in______ leads to the accumulation of sphingomyelin in the macrophages.
sphingomyelinase
Sea-blue histiocytosis is caused by unknown deficiency. _________ are found in the spleen and bone marrow
Sea-blue macrophages
Tay-Sachs disease is characterized by deficiency in ______ enzyme
Hexosaminidase A
Tay-Sachs disease is characterized by deficiency in a certain enzyme which leads to the accumulation of ______ and ______ exhibited by vacuolated cytoplasm
glycolipids
gangliosides
Sand Hoff’s disease is characterized by deficiency in _____ enzyme which leads to the accumulation of glycolipids and gangliosides exhibited by vacuolated cytoplasm
Hexosaminidase A & B
Characterised by having decreases in the absolute number of monocytes associated with stem cell disorders such as aplastic anemia
Monocytopenia
Epstein-Barr virus (EBV) infects _______
B lymphocytes.
Infectious mononucleosis is common in the 14-24 age group with symptoms ranging from
malaise and fever
pharyngitis
lymphadenopathy
splenomegaly
Infectious mononucleosis is transmitted through ___
nasopharyngeal secretions
Infectious mononucleosis is ____ for heterophile antibody test
positive
Symptoms similar to infectious mononucleosis and is transmission is by blood tranfusions and saliva exchange.
Cytomegalovirus (CMV)
In Cytomegalovirus (CMV), _____% of lymphocytes can be reactive.
90
Cytomegalovirus (CMV) is _____ heterophile antibody test
negative
Associated with adenovirus and coxsackie A virus
Infectious lymphocytosis
Contagious disease mostly affecting young children
Infectious lymphocytosis
Lymphocytosis with no reactive lymphocytes
Infectious lymphocytosis
Other Conditions Associated with Lymphocytosis (viral or non viral):
hepatitis
Viral
Other Conditions Associated with Lymphocytosis (viral or non viral):
influenza
Viral
Other Conditions Associated with Lymphocytosis (viral or non viral):
mumps
Viral
Other Conditions Associated with Lymphocytosis (viral or non viral):
measles
Viral
Other Conditions Associated with Lymphocytosis (viral or non viral):
rubella
Viral
Other Conditions Associated with Lymphocytosis (viral or non viral):
varicella
Viral
Other Conditions Associated with Lymphocytosis (viral or non viral):
Bordetella pertussis (whooping cough)
Nonviral
Other Conditions Associated with Lymphocytosis (viral or non viral):
brucellosis
Nonviral
Other Conditions Associated with Lymphocytosis (viral or non viral):
toxoplasmosis
Nonviral
Atypical Lymphocytes also referred to as reactive/variant/stimulated lymphocytes or _______
DOWNEY CELLS
Also called as Turk’s irritation cell which is actually a plasmacytoid lymphocyte with large black chromatin
Type I Downey Cell
Found in infectious mononucleosis with round mass of chromatin (ballerina skirt appearance)
Type II Downey Cell
Vacuolated lymphocyte resembling a swiss cheese or moth-eaten appearance
Type III Downey Cell
Originally B cells with hair like projection which are identified by being TRAP resistance
Hairy Cell
Destroyed lymphocyte
Basket cell
Basket cell also called as ____ (thumbprint appearance)
smudge cells
Lymphocyte usually abundant in Chronic Lymphocytic Leukemia (CLL)
Basket cell
Lymphocyte with cerebriform nucleus (Brain-Like)
Sezary Cells
Lymphocyte usually seen in mycosis fungoides and Sezary syndrome
Sezary Cells
plasma cell with red to pink cytoplasm; associated with increased IgA and usually seen in Multiple Myeloma
Flame cell
plasma cell with vacuoles; with large protein globules called as “Russell Bodies”
Grape Cell/Berry/Morula/MOTT Cells