Non-malignant WBC Disorders Flashcards by Kimberly Meinecke

Neutrophilia

Increased neutrophils >7.0 x 10^9/L
Not pathogenic (usually)
Exercise, anesthesia, newborn

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Pseudoneutrophilia

No increase in bands
Redistribution of MGP and CGP

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Acute Neutrophilia

Bacterial infection/toxin
Bone marrow response (shift left)
10-20 K

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Chronic Neutrophilia

Continuous stimulation
Bone marrow storage depletion
Toxic granulation with Dohle bodies
Bacterial infection

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Leukemoid Reaction

Extreme reaction to severe infection
Necrotic tissue
Usually >25k
Many immature cells

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Leukemoid Reaction Lab Results

Normal RBC
Normal Platelet
Toxic granulation
Dohle Bodies
LAP increased

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Neutrophilia Other Causes

Tissue necrosis
Burns
Rheumatoid arthritis
Tumors
Steroids
Pregnancy

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Neutropenia

<2.0 x 10^9/L of 1.5 x 10^9/L
Decreased Bone Marrow production
Stem cell disorders

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Neutropenia Causes

Radiation
Chemotherapy
Myleophthisic anemia
Megaloblastic anemia
Inherited disorders

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Increased WBC loss

Severe infection
Immune loss
Hypersplenism
Hemodialysis
Viral infection

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Pseudoneutropenia

Increase in MGP
Transient

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Agranulocytosis

<0.5 x 10^9/L
Possibility of severe infection

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Chronic Granulomatous Disease

Inherited
Abnormal Oxidative metabolism
Recurrent infections
Nitroblue tetrazolium test (NBT)

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Chediak Higashi Anomaly

Inherited
Fusion of primary and secondary granules
Abnormal lysosomes
Hypopigmentation
Death in infancy

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May-Hegglin Anomaly

Inherited
Large basophilic inclusions in cytoplasm
+/- Thrombocytopenia
+/- Bleeding

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Pelger-Huet Anomaly

Benign, Inherited
Hyposegmentation (bi-lobed segs and inc bands)
Pince nez cells (old spectacle look)
Normal Cell function

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Alder Reilly Anomaly

Inherited
Large, purple granules in WBC cytoplasm
Enzyme deficiency
Cells appear as clusters and may have vacuoles
Normal cell function

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Myeloperoxidase Deficiency

Inherited
No increase in infection - alternative methods to kill bacteria
Causes technical error with instruments

Leukocyte Adhesion Deficiency

Inherited
Absence of WBC surface adhesion proteins (integrins)
Defects in adhesion, chemotaxis, phagocytosis, degranulation
Frequent infections
High mortality

Systemic Lupus Erythematosis (SLE)

Connective Tissue Disease
Anti DNA acts on WBC (LE factor)
Round, homogenous body ingested by neutrophil (LE cell)
Butterfly rash
ANA testing

Eosinophilia

> 450/cumm
Parasitic infection
Allergy
Asthma
Hypersensitive Skin Disease
Hematologic Malignancy
Tryptophan

Hypereosinophilia Syndrome

> 1500/cumm
Tissue infiltration and damage
Charcot Leyden crystals
Nasal and Paranasal sinus contents - allergic rhinitis

Eosinopenia

Hard to define
Cushing’s syndrome - bodies produce too much cholesterol

Basophilia

> 150/cumm
Immediate hypersensitivity reactions and MPD
Malignancy

Basopenia

Difficult to establish Due to inflammation and immunologic reactions

Monocytosis

>800/cumm Inflammatory conditions and malignancies Recovery stage of acute infection and agranulocytosis Immune response to TB Collagen vascular disorders Fungal infections

Monocytopenia

<200/cumm Hard to establish Stem cell disorders

Lipid Storage Disorders

Deficiency in enzyme to phagocytose material

Gaucher's Disease

Accumulation of lipid in macrophages Deficiency in B-glucocerebrosidase Pancytopenia Splenomegaly, Bone pain Increased serum phosphatase

Nieman-Pick Disease

Ashkenazie population Deficiency of spingomyelinase Poor physical development Foamy macrophages Often fatal by 3 yrs old

Tay-Sachs Disease

Ashkenazie population Deficiency of B-hexoseamidase Accumulation of glycolipids & mucopolysaccharides Affects CNS Often fatal by 4 yrs old

Sea Blue Histiocyte Syndrome

Inherited - Benign Splenomegaly Decreased platelets Sea Blue staining macrophages in spleen and bone marrow

Lymphocytosis

Adenovirus or Coxsackie A virus Respiratory infection Fever Headache Dizziness Painful Neck

Lymphocytic Leukemoid Reaction

Increase in WBCs and Lymphs Extreme response to viral infection Reactive and immature lymphs

Plasmacytosis

Plasma cells (make up 4% of Bone Marrow) Mott Cell with Russell Bodies Flame Cells

Infectious Mononucleosis

Epstein-Barr Virus 14-24 yr olds Infects B-cells Many reactive lymphs Monospot Test

Cytomegalovirus (CMV)

Herpes group virus Most common viral infection Dangerous to newborns

Lymphocytopenia

Inability to mount response to infection Steriods Acute inflammation infections Cancer SLE Chemotherapy/radiation Malnutrition

Acquired Immune Deficiency Syndrome

Pancytopenia Decreased lymphocytes Decreased Th cells (CD4)

Severe Combined Immune Deficiency Syndrome (SCIDS)

Deficiency of T and B cells Decreased lymphoid tissue in nodes and bone marrow Recurrent infection Rash Failure to Thrive Death to overwhelming sepsis within 2 yrs of life

Wiskott-Aldrich Syndrome

Decreased antibodies Eczema, thrombocytopenia, immune deficiency Death by age 10 due to infection/bleeding

DiGeorge Syndrome

Decreased T cells and lympoid tissue Normal B cells No thymus (hypoparathyroidism) Heart defects, hypocalcemia

Bruton's Disease

Agammaglobulinemia Decreased B cells and plasma cells Normal T cells Respiratory and skin infections with pyogenic bacteria

LAP scores

CML <13 Normal, healthy 13 - 130 Leukomoid Reaction >160