HEMA1LEC LESSON 9 - Quantitative and Qualitative Disorders of Granulocytes and Monocytes PDF

[TRANS] LESSON 9: QUANTITATIVE AND QUALITATIVE DISORDERS OF WBC DISORDERS OF GRANULOCYTES AND o Occur during physiologic conditions such as exercise. MONOCYTES o Occur in Leukocytosis → increased in WBC QUANTITATIVE DISORDERS...

[TRANS] LESSON 9: QUANTITATIVE AND QUALITATIVE DISORDERS OF WBC DISORDERS OF GRANULOCYTES AND o Occur during physiologic conditions such as exercise. MONOCYTES o Occur in Leukocytosis → increased in WBC QUANTITATIVE DISORDERS OF GRANULOCYTES Rate of outflow from the blood to the tissues AND MONOCYTES o From circulation to tissues o Leukocyte’s function is to carry out their response to Quantitative Disorders → refers to quantity or number stimuli in tissues and only pass through circulation. Increase or decrease number or proportions of WBC types and Leukocyte count Involved if the disorder is “cytosis” or “penia” LEUKOCYTOSIS o Increased (↑) → suffix “philia” or “cytosis” Leuko → WBC; cytosis → increase in quantity. o Decreased (↓) → suffix “penia” Absolute increase in the concentration of leukocytes higher than normal for a certain age. Greater than 11 x 109 /L (adults) NONPATHOLOGICAL FACTORS THAT AFFECTS o Updated reference value → 4.5 – 11 x 109 /L WBC COUNT: o Some references → 4.5 – 11.5 x 109 /L Age of the patient – adult (4.5 – 11.0 109 / ul) vs newborns o Since it varies, leukocytosis is considered if the WBC (18 x 109 / ul) count is >11.5 (greater than the upper limit of reference o NOTE: reference range of hospitals varies since there range) is no definite/standard WBC reference range. Demargination; increase BM production and inflow Patient’s basal condition – physical and mental relaxation NOTE: Watch the recorded lecture for better vs exercise and stress understanding. o If there is physical or mental relaxation, the overall WBC count can be decreased to normal. Table 1. Example Scenario of o If there is stress and exercise, it can trigger increase Relative and Absolute Neutrophilia amount of WBC count. Relative Value Absolute Value Physiological conditions – convulsions, electric shock Neutrophils Normal: 40 – 70% Normal: 2- 7.7 and pregnancy o These factors can decrease the WBC count Relative Absolute Neutrophilia: 75% Neutrophilia: 9.0 CATEGORIES OF MECHANISM THAT CAUSES o When there is a “relative” neutrophilia, only the relative QUANTITATIVE ALTERATIONS – FOR EVALUATING value is increased LEUKOPENIA, LEUKOCYTOSIS o When there is a “absolute” neutrophilia, only the absolute value is increased Leukopenia → decreased in WBC count Relative → the only affected is the specific cell line Leukocytosis → increased in WBC count (Neutrophils) Rate of production (BM) and inflow of cells to the Absolute → It can affect the overall/total WBC count. peripheral blood (circulation) Leukocytosis → absolute increase in WBC, because there o Increase production or not able to compensate what is an absolute increase in specific WBC has been lost to tissues. o ↑ in production = ↑ number of neutrophils if cells are effectively released in the circulation. NEUTROPHILIA o NOTE: same mechanism to RBC Greater than 7.8 x 109 (adults) with band cells and ▪ Depends on Bone Marrow capacity metamyelocytes which are indications of marrow ▪ How many percent of cells reach in circulation → compensation. presence of effective and ineffective Accompanied with shift to the left (Increase appearance of hematopoiesis immature cell in the circulation) Distribution between the circulating (CLP) and Occurs during 2nd phase of inflammation marginal pools (MLP) MOST COMMON CAUSE OF LEUKOCYTOSIS o Should always have equilibrium between CLP and MAIN FACTOR: Neutrophil is the most abundant type of MLP WBC in the circulation ▪ Neutrophil → 1 (CLP):1 (MLP) NORMAL RELATIVE PERCENTAGE: 50% TO 70% ▪ Monocyte → 1 (CLP): 3.5 (MLP) ABSOLUTE COUNT: 2-7.7 X 109/ul o The ones that easily migrate to tissues are those CAUSE OF NEUTROPHILIA: adhering to walls replaced by leukocytes in CNP. o SYSTEMIC BACTERIAL INFECTIONS ▪ Demargination of neutrophils and increased Margination – leukocytes in CNP shift to marginal pool inflow into the circulation; pyogenic infections o Decreased cells in circulation o TOXIC FACTORS o CNP → Neutrophilic ▪ Metabolic conditions associated with the o CLP → Leukocytes (refers to other WBC as well) production of substances or accumulation of o Occur in cases of Leukopenia metabolic products, drugs and chemicals Demargination – leukocytes in marginal pool shift to the toxic to bodies circulating pool A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 1  TRANS: QUANTITATIVE AND QUALITATIVE DISORDES OF WBC ▪ METABOLIC DISEASES: Gout, Uremia, MONOCYTOSIS Diabetic ketoacidosis Infections – TB, Subacute bacterial endocarditis (SBE), ▪ DRUGS: Injections such as ACTH, Growth syphilis, protozoan and rickettsial infections Hormone, Epinephrine and Steroids; CONVALESCENCE – constitutes the 2nd wave of Chemicals such as Pb and Hg. granulocytes o LEUKEMOID REACTION Inflammatory conditions ▪ Non leukemia leukocytosis characterized by a shift to the left with increased band forms Myeloproliferative disorder and few metamyelocytes or myelocytes but Autoimmune conditions – systemic lupus erythematosus, no myeloblast in the peripheral blood Rheumatoid arthritis and sarcoidosis. ▪ Associated with Pneumonia, Meningitis, NOTE: If there is a significant increase in the absolute monocyte Tuberculosis and in cases of severe count (>1.0 × 10⁹/L) hemolysis or metastatic carcinoma ▪ Marked increased in number of leukocytes QUANTITATIVE DISORDERS OF GRANULOCYTES greater than 30 x 109/L (High WBC Count) AND MONOCYTES ▪ Leukocyte Alkaline Phosphatase (LAP) or Leukopenia Neutrophil Alkaline Phosphatase (NAP) o Reduction of the absolute count to lower than both are cytochemical test used to 3000/mm3 or 4,500/mm3 differentiate leukemoid from CML (Chronic o Ineffective production; Margination; Increase outflow, Myelogenous Leukemia) Decrease survival. o TISSUE DESTRUCTION OR NECROSIS: NOTE: Ineffective production, margination, increased outflow, ▪ Myocardial Infarction, Burns, Surgery, Crush and decreased survival can all lower the total WBC count. Injuries, Fractures, Massive hemolysis Margination refers to the movement of WBCs from circulation to o MYELOPROLIFERATIVE DISORDER tissues, while decreased survival results from ineffective o PSEUDONEUTROPHILIA hematopoiesis or leukopoiesis. 3 PHASES OF INFLAMMATION: o 1ST PHASE: Transient neutropenia during early phase LEUKOPENIA of infection (acute infection) due to shift of leukocytes NEUTROPENIA from CNP to MNP. Decrease of neutrophils 1. DECREASED OR INEFFECTIVE PRODUCTION o 2nd PHASE: Increase in production in the bone marrow FANCONI’S ANEMIA and rate of inflow in circulation o inherited bone marrow failure syndrome characterized o 3rd PHASE: Convalescence, otherwise known as by aplastic anemia RECOVERY PERIOD. o inherited hypoplasia; indistinguishable with aplastic anemia EOSINOPHILIA o Decreased/absence of production Increase in number of eosinophils KOSTMANN’S SYNDROME Neutralize mast cell and basophil secretions associated o infantile genetic (inherited) agranulocytosis with allergic or hypersensitivity reactions o Decreased/absence of production o Allergic Diseases (hypersensitivity): Urticaria, hay CYCLIC NEUTROPENIA fever/seasonal rhinitis, asthma o recurrent episodes of severe neutropenia and o Parasitic Infections: Trichinosis, Schistosomiasis, therefore, symptomatic infection Cutaneous larva migrans, Toxocariasis, o Transient in contrast to Fanconi and Kostmann Cysticercosis o Decreased/absence of production o Infectious Disease: Scarlet fever, Eosinophilic NOTE: Neutropenia, the opposite of neutrophilia, occurs when fasciitis the absolute neutrophil count falls below 2.0 x 10⁹/L. This results o Pulmonary Eosinophilias: Loffler’s syndrome from decreased or ineffective production, similar to red blood o Non-parasitic Infections: caused by systemic cells (RBCs), where despite bone marrow production, they do fungal infections and chlamydial agents not enter circulation effectively. o Myeloproliferative Disorders MYELOPHTHISIS (low stem cells low production) o Drugs: Pilocarpine, Digitalis RADIATION o Idiopathic Hypereosinophilic Syndromes o causes destruction of progenitor cells (unknown cause of eosinophilia) DRUGS AND TOXINS ABSOLUTE EOSINOPHILIC COUNT: > 0.4 x 109/L o CA chemotherapy, chloramphenicol, antibiotics, alcohol, benzene, anti-folate drugs and heavy metal BASOPHILIA MYELOKATHEXIS Allergic diseases o congenital disorder characterized by inability to release Viral infections mature granulocytes into the blood Chronic hemolytic anemia (e.g., varicella (chicken pox or INTRAMEDULLARY DESTRUCTION (e.g., Chédiak– small pox) Higashi syndrome and megaloblastic anemia) Inflammatory conditions (e.g., ulcerative colitis) 2. Decreased survival of neutrophils (Rate of Outflow) Myeloproliferative disorders EXHAUSTIVE CHRONIC (LONG TERM) INFECTIONS o marrow becomes depleted and production cannot keep NOTE: Basophils increase in the bloodstream to mediate up with peripheral utilization. hypersensitivity reactions. Though they have some VIRAL INFECTIONS phagocytic ability, it is limited. An absolute basophil count o transient neutropenia (decrease in neutrophils) occurs. above 0.15 x 10⁹/L is defined as basophilia. HYPERSPLENISM o selective removal of neutrophils by spleen. A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 2  TRANS: QUANTITATIVE AND QUALITATIVE DISORDES OF WBC IMMUNE NEUTROPENIA – immune- associated destruction associated with collagen vascular disease, Rheumatoid Arthritis (Felty’s syndrome), SLE, AIDS. EOSINOPENIA o In Felty’s syndrome, chronic neutropenia and It is defined as a decrease in the absolute neutrophil count splenomegaly occurs. to less than 0.09 x 10⁹/L. 3. ALTERED DISTRIBUTION BETWEEN THE CIRCULATING POOL AND THE MARGINAL POOL Acute stress Acute infection - transient neutropenia due to the acute ACTH and epinephrine secretion migration of neutrophils from the MLP into the tissues and Acute inflammatory or infectious states shift of cells from the CLP into the MLP. o 1st Phase of Inflammation; initial wave of neutrophils to the tissues. BASOPENIA PSEUDONEUTROPENIA - due to the margination of It is defined as a decrease in the number of basophils, neutrophils. which is difficult to detect due to their low relative o Ex. Presence of endotoxin causing the shifting. percentage in the blood. Detection is possible only when ▪ This is a non-pathological condition, as it does not using the absolute basophil count. reflect the underlying pathological cause for the Hormones – corticotropin, progesterone, ACTH decrease in neutrophils in the blood. During ovulation – increase progesterone ▪ Thyrotoxicosis SUMMARY OF MECHANISMS MONOCYTOPENIA LEUKOCYTOSIS/NEUTROPHILIA NEUTROPENIA It occurs when the absolute basophil count is less than 0.2 ↑ rate of production and release or ↓ rate of production x 10⁹/L and is considered a rare condition. inflow from BM to circulation and release or Steroid therapy ineffective production Epstein-Barr Virus (EBV) infection Demmargination Margination (1st phase Hairy Cell Leukemia in acute infection) ↓ rate of outflow, ↑ survival of ↑ rate of outflow from QUALITATIVE DISORDERS OF GRANULOCYTES granulocytes and monocytes circulation to tissues, AND MONOCYTES (stayed in circulation for a long time) ↓ survival in the circulation "Qualitative" refers to abnormalities in both the structure and function of cells, indicating that there is an issue with how the cells are built and how they operate. TABLE: CYTOPLASMIC AND NUCLEAR ABNORMALITIES ALDER-REILLY TOXIC GRANULATION ANOMALY / DOHLE (derʹlĕh) BODIES MAY-HEGGLIN PARAMETER GRANULES ANOMALY APPEARANCE DESCRIPTION Prominent, dark Dense purple- Single or multiple Pale blue Dohle blue-purple red particles in small, oval inclusions body-like granules, either fine all WBC types in the periphery of inclusions in or heavy, in resembling neutrophils that stain granulocytes and metamyelocytes, coarse toxic pale blue with Wright’s monocytes band, or granules. stain Larger, more segmenters (shift to Permanent Occur along with toxic prominent the left) granules PAS (–) POD (+) PAS (+) (peroxidase) Transient CHANGE/ Azurophilic granules Abnormal deposition Remnants of RNA (rRNA) Structural RNA (mRNA) DEFECT that have retained their and storage of and free ribosomes or alterations basophilic qualities mucopolysaccharide rough ER during accelerated s. granulopoiesis DISEASE STATE Severe infections Genetic Scarlet fever Rare autosomal and toxic abnormality Burns dominant disorder conditions. associated with Chronic infections associated with ↑ demand for mucopolysacch (can also have toxic mild neutrophils aridoses. granulation and dohle thrombocytopenia bodies in one cell) (decrease platelet) Aplastic anemia Giant platelets Following exposure to Slight bleeding toxic drugs and tendency chemicals A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 3  TRANS: QUANTITATIVE AND QUALITATIVE DISORDES OF WBC CHEDIAK-HIGASHI AUER RODS LE CELL TART CELL PARAMETER ANOMALY APPEARANCE DESCRIPTION Gigantic, coarse, Linear or spindle- PMN (granulocytic Neutrophil or irregular granules shaped red-purple cell) with an monocyte with an in the cytoplasm of inclusions in ingested round ingested nucleus of granulocytes and myeloblasts and body that appears a lymphocyte monocytes monoblasts purplish, POD (+) MPO, Sudan, homogenous, Blood and marrow CAE, ALP (+) amorphous round smear (AML) mass found Blood and Bone centrally marrow smears CHANGE/ Abnormal lysosomal Aggregated primary Engulfment of the Nucleophagocytosis DEFECT development granules caused by nuclear material of abnormal development another PMN or a of lysosomes lymphocytes DISEASE STATE Autosomal Acute myelocytic Lupus No diagnostic recessive disorder leukemia erythematosus significance that presents with Chronic recurrent infections myelomonocytic Abnormal leukemia (CMML) pigmentation Neuropathy Photophobia GAUCHER’S DISEASE NIEMANN-PICK DISEASE JORDAN’S ANOMALY PARAMETER (Gaucher Cell) APPEARANCE DESCRIPTION Monocytes and “Foam-cell” appearance of Abundant lipid-containing macrophages have monocytes and vacuoles in the cytoplasm eccentric nucleus and macrophages of granulocytes and wrinkled cytoplasm that PAS (–) lymphocytes, in the blood has a pale onion-skin and marrow appearance Sudan (+) PAS (+) Lipid containing inclusion bodies CHANGE/ Accumulation of Deficiency in the enzyme Familial vacuolization of DEFECT glycosphingolipids due to (Sphingomyelinase) that leukocytes deficiency of cleaves phosphoryl choline from β-glucocerebrosidase sphingolipids DISEASE STATE Lipid storage disease Lipid storage disease Genetic disorder (Lipidoses) (Lipidoses) Related to lipidoses – abnormal deposition and storage of vacuoles PARAMETER APPEARANCE DESCRIPTION CHANGE/DEFECT DISEASE STATE Nuclei of Deficiencies of vitamin Associated with neutrophils have B12 and folic acid megaloblastic HYPERSEGMENTATI five or more lobes anemia or a ON Asynchronous hereditary disorder (NUCLEAR) N:C growth Inherited – Undritz’ Anomaly A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 4  TRANS: QUANTITATIVE AND QUALITATIVE DISORDES OF WBC Nuclei of Failure of normal Autosomal neutrophils are nuclear segmentation dominant condition bilobed, or are band-shaped, peanut-shaped, or PELGER-HUET pince nez-shaped; ANOMALY nuclear chromatin (NUCLEAR) is coarse and clumped Hypo-segmented Pseudopelger-Huet Anomaly Pseudo – means false Associated with hematologic neoplasm and other disorder More on malignant cells. FUNCTIONAL DEFECTS QUANTITATIVE DISORDERS OF IMMUNE ⚫ Refers to WBC function LEUKOCYTES LYMPHOCYTOSIS LAZY LEUKOCYTE SYNDROME ⚫ Absolute circulating increase in the lymphocyte count > ⚫ Granulocytes do not respond to chemotactic factors than 4800/ul (adults), 7000/ul (children), 9000/ul (infants) and therefore, fail to accumulate at the inflamed tissue. ⚫ The number of lymphocytes decreased with age; infants ⚫ Delayed or impaired chemotaxis. with highest circulating lymphocytes ⚫ They are not being attracted at the chemotaxins (LYMPHOCYTOPENIA). o It is normal for infants/newborns to have higher circulating lymphocytes. CHRONIC GRANULOMATOUS DISEASE o Adults: 20-40% ⚫ Granulocytes are capable of phagocytosis and o Neonates: 50%-70% degranulation but are incapable of the subsequent ⚫ In viral infections, in which it cause increase in bacterial killing process due to a decrease in the lymphocytes; may or may not be accompanied by variant production of H2O2 lymphocyte. ⚫ Neutrophil or monocyte are capable of ingestion and ⚫ If phagocytes are more on against bacterial, fungal, and release of components of their granules. However, they parasitic infections, lymphocytes are more on against are not capable of killing process due to lack or none of viral infections. H2O2. ⚫ It might be absolute lymphocytosis where even total ⚫ H2O2 is necessary in oxygen dependent killing. It is toxic leukocyte count will be high or relative lymphocytosis and bactericidal. where percentage is high but total leukocyte count is not ⚫ Impaired killing, impaired superoxide dismutase. increased or normal o Superoxide dismutase - an enzyme that is also ⚫ Relative count - High lymphocytes, but total WBC count is included in oxygen dependent killing not affected ⚫ Clinically characterized by frequent infections, enlagred ⚫ Absolute lymphocytosis - High lymphocytes, total WBC is spleen and liver, lymphadenitis, and granulomas altered and affected ⚫ To test CGD, we use Nitroblue Tetrazolium Test o Result is Weak Staining INFECTIOUS LYMPHOCYTOSIS ⚫ Occurs mainly in children ⚫ Caused by viruses (echovirus, adenovirus) GLUCOSE-6-PHOSPHATE DEHYDROGENASE ⚫ Laboratory findings include a leukocytosis of 20,000- DEFICIENCY 50,000/mm3 (absolute), 60-95% lymphocytes (relative ⚫ G6PD is part of Hexose Monophosphate Pathway percentage) ⚫ If there is G6PD deficiency, there is inability to produce a ⚫ Predominance of lymphoid cells and presence of younger respiratory burst due to impaired HMP. forms with neutrophilia (shift to the left); can also have features that are typical of infectious stage and toxic MYELOPEROXIDASE DEFICIENCY conditions. ⚫ Myeloperoxidase is part of granular content and important in manner of killing PERTUSSIS ⚫ If there is MPO deficiency, there is impaired bacterial ⚫ Caused by Bordatella pertussis killing ⚫ Laboratory findings include a lymphocytosis of as high as ⚫ MPO is seen in primary phagocytic cells, neutrophils, and 30,000/mm3 (absolute lymphocytosis) monocytes. INFECTIOUS MONONUCLEOSIS ⚫ Mimics findings associated with Infectious Lymphocytosis with 60-90% lymphocytes but with 12,000-25,000/mm3 A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 5  TRANS: QUANTITATIVE AND QUALITATIVE DISORDES OF WBC leukocyte count; neutrophilia with a left shift, toxic o Note: Variant Lymphocytes, overall size is larger than granulation, and Dohle bodies. normal lymphocyte. They are also known as reactive ⚫ Causative agent: Epstein Barr Virus lymphocytes. o Variant/Reactive/Atypical Lymphocytes are seen in CYTOMEGALOVIRUS INFECTION disorders such as infectious mononucleosis, viral pneumonia, viral hepatitis. They are called Reactive ⚫ Characterized by leukocytosis (absolute increase) Lymphocytes because they only appear due to involving a lymphocytosis ( relative) in which 20% or more response to an antigenic stimulus (appearance of of lymphocytes may be reactive. certain virus) o Remember! Atypical Lymphocytes are called TOXOPLASMOSIS Abnormal Lymphocytes because they are seen in ⚫ Relative lymphocytosis and variant, atypical lymphocytes ABNORMAL CONDITION but they still function as on the peripheral blood smear. NORMAL LYMPHOCYTES. ⚫ Relative because lymphocytes are only increased, but total Nucleus may be enlarged and may be lobulated, WBC count is unaffected. monocytoid, or folded; chromatin patterns vary from fine to coarse; 1-3 nucleoli may be present (may or may not have MISCELLANEOUS CAUSES nucleoli). ⚫ Viral and non-viral disorders, and drugs Cytoplasm is frequently abundant and often foamy or ⚫ Viral: Measles, Mumps, Rubella, smallpox, chickenpox vacuolated; gray to light blue or intensely blue in color; may ⚫ Non-viral: Bacterial and parasitic infections sich as contain granules. diptheria, TB, typhus, Rickettsiae, Brucellosis, malaria, o Note: There is a peripheral basophilia or radial syphilis. basophilia. There is an irregular shape and presence ⚫ Drugs: Para-aminosalicylic and phenytoin hypersensitivity of vacuole. LYMPHOCYTOPENIA Refers to the condition of decreased lymphocyte count or decreased in the absolute count of 5% of total leukocytes) A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 6  TRANS: QUANTITATIVE AND QUALITATIVE DISORDES OF WBC 2. RIEDER CELLS 2. WALDENSTROM’S MACROGLOBULINEMIA ⚫ Similar to normal lymphocytes except that the nucleus is Always associated with lymphocytosis notched, lobulated, and cloverleaf-like Generalized proliferation of B cells and plasma cells and an ⚫ Occur in CLL (Chronic Lymphocytic Leukemia) or can be increase (↑) of monoclonal IgM in serum artificially produced through blood smear preparation Laboratory profile includes: lymphocytosis, IgM level >1.0 g/dL (>15% of total amount of serum proteins ), N/N 3. VACUOLATED LYMPHOCYTES anemia, thrombocytopenia or pancytopenia, marked ⚫ Frequently associated with lipid storage diseases such as rouleaux formation (greater degree), and increased ESR Niemann-Pick disease and Tay-Sachs disease, Hematologic feature: pancytopenia due to BM infiltrations mucopolysaccharidoses diseases such as Hurler (uncontrolled proliferation of B cells and Plasma cells); syndrome, and Burkitt lymphoma there is inappropriate differentiation of plasma cells When the normal BM parenchyma becomes 4. SMUDGE CELLS replenished by abnormal/proliferative cells will cause ⚫ Natural artifact produced in the preparation of a blood hypoplasia due to decrease in number of precursor cells smear (trauma) (pancytopenia) ⚫ Represent the bare nuclei of lymphocytes (paler Thrombocytopenia clumping of platelets due to IgM staining property) Gammopathy (gamma spike) in electrophoresis that ⚫ Seen in increased numbers in lymphocytosis particularly represents increase concentration of IgM in serum CLL NOTE: If artifactual or smear preparation occurs (such as with 3. HEAVY-CHAIN DISEASES basket cells or smudge cells), WBCs can be damaged. The ⚫ Disorders related to the production and excretion of the difference between the two is that the cytoplasmic appearance immunoglobulin heavy chains without the light chains of basket cells is basket-like in granulocytes, while smudge cells ⚫ Increased (↑) of gamma (IgG), alpha (IgA), mu (IgM) are associated with lymphocytes. PRIMARY IMMUNODEFICIENCY DISORDERS: 5. HAIRY CELLS PURE B CELL DEFICIENCY ⚫ Lymphocyte with hair-like cytoplasmic projections ⚫ Affected lymphocytic type of B cell. ⚫ Seen in hairy cell leukemia ⚫ Tartrate Resistant Acid Phosphatase (TRAP) + INFANTILE X-LINKED (BRUTON’S) 6. SEZARY CELLS AGAMMAGLOBULINEMIA ⚫ Developmental defect of B cells that primarily affects ⚫ Convoluted nucleus which is described as cerebriform male infants; females are carriers (pass by mothers to ⚫ Seen in the leukemic phase of Mycosis fungoides infants) ⚫ B cell zones of lymph nodes and spleen are depleted of B 7. GRAPE OR MOTT CELLS cells ⚫ Cytoplasm is completely filled with Russell bodies which ⚫ Seen during period of infancy with recurrent infections due are acidophilic refractile bodies which represents to absence of B cell zones in lymph nodes and spleen gammaglobulins in the cytoplasm of plasma cell o Russell bodies if there are only a few acidophilic HYPOGAMMAGLOBULINEMIA OF INFANCY refractile bodies. If they are abundant, they are referred ⚫ Decrease in immunoglobulins caused by delayed to as grape or mott cells. synthesis in the first years of life ⚫ Affected infants are subject to recurrent infections 8. FLAME CELLS ⚫ Self-correction during 2 years of age. ⚫ Cytoplasm stains a bright-red color and contains increased quantities of glycogen or intracellular LATE-ONSET VARIABLE PRIMARY deposits of amorphous matter (inclusions) HYPOGAMMAGLOBULINEMIA ⚫ Occurs in adults usually by 30 years of age defect in the 9. SNAPPER-SCHNEID BODIES differentiation of B lymphocytes into plasma cells ⚫ Inclusions found in the cytoplasm of plasma cells after ⚫ Patients have an increased occurrence of infections and therapy with amidine drugs - following treatment of MM an increased incidence of autoimmune disorders. HYPER-GAMMAGLOBULINEMIA SELECTIVE IMMUNOGLOBULIN DEFICIENCY ⚫ Increased (↑) Immunoglobulins in blood which affects WBC ⚫ Acquired decrease in one subtype of immunoglobulin, ⚫ Level of one or more serum immunoglobulins are commonly IgA increased. ⚫ Different immunoglobulins: IgG, IgA, IgM, IgE, IgD PRIMARY IMMUNODEFICIENCY DISORDERS: 1. MULTIPLE MYELOMA PURE T CELL DEFICIENCY ⚫ Monoclonal gammopathy in which only one type of ⚫ Affected lymphocytic type of T cell. gammaglobulin is increased ⚫ Laboratory profile includes: N/N (normocytic / THYMIC APLASIA normochromic) anemia, normoblasts on the peripheral ⚫ Thymic aplasia/hypoplasia blood smear rouleaux formation and increased ESR, ⚫ Developmental defect of the thymus gland circulating plasma cells, “M-spot” in electrophoresis, ⚫ T cell zones of the lymph nodes are depleted/absent increase concentration of Bence-Jones Protein in plasma ⚫ Acquired Type and urine ⚫ ↑ in IgG = due to increase of plasma cells NEZELOF’S SYNDROME ⚫ This is not associated with Lymphocytosis. ⚫ Thymic aplasia/hypoplasia (also has T cell depletion) ⚫ Genetic Type A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 7  TRANS: QUANTITATIVE AND QUALITATIVE DISORDES OF WBC ⚫ Autosomal recessive disorder PRIMARY IMMUNODEFICIENCY DISORDERS: SEVERE COMBINED IMMUNE DEFICIENCY ⚫ Affected both B and T cells SWISS TYPE COMBINED ⚫ Loss of T and B cell function ⚫ Little or to no Ig production WISKOTT-ADLRICH SYNDROME ⚫ Failure of T cell response = T cell ⚫ Absence of IgM production = B cell ATAXIA TELANGIECTASIA ⚫ Characterized by ataxia or loss of muscle coordination ⚫ Telangiectasia – dilation of smaller blood vessels that may predispose patient to bleeding ⚫ No IgA that is why patient is predispose to upper recurrent respiratory tract infections o Both B and T cell are affected because there is a decrease in IgA, possible decrease in IgE, and has thymic aplasia REFERENCES Notes from the discussion by Prof. Charmaine P. Peralta, RMT, MSMT National University powerpoint presentation: Prof. Charmaine P. Peralta, RMT, MSMT A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 8  TRANS: QUANTITATIVE AND QUALITATIVE DISORDES OF WBC A. BAGOOD, S.A. BURGOS, K.D. JUDAL, R.A. LUZADAS, A.J. MANGILIT, S.M. REAMBONANZA, S.C. TUMAGAY, N.T. VELASCO | BSMT 9