Myeloid Line

Toxic Granulation Persistent staining of primary granules

Toxic Vacuolation Indicates phagocytic activity and deganulation
Dohle bodies RNA remnants
Shift to the Left increase myelo, meta, stabs @ pb
Functional Disorder
Chronic Granulomatous Dse granules fail to degranulate
Chediak- Higashi Syndrome abnormal fusion of primary and secondar granules
Nuclear Disorder
Hypersegmention
Pelger-Huet Anomaly nucleus does not mature past 2 lobes, peanut shaped
Pseudo Pelger-Huet nucleus appears round
Cytoplasmic
May-Hegglin Anomaly has crystalline cigar-shaped Dohle-like incusions
Alder-Reily Anomaly azurphilic granules in cytoplasm of all or only one cell line
Lipid Storage Disorders for Monocytes
Gauchers Disease deficiency in glucocerebrosidase causing glucocerebroside to accumulate
Niemann- Pick Disease deficiency in sphingomyelinase causing sphingomyelin to accumulate in
Sea-blue Histiocytosis unknown deficiency. Sea-blue colored macrophages
Non-malignant Lymphocytosis
Infectious mononucleosis Epstein-Barr virus infects B cells ; (+) heterophile Ab; T lymph attacks affected
B cells
Cytomegalovirus symptoms same with Infectious mono but (-) heterophile Ab
Infectious lymphocytosis assoc. With adenovirus and Coxsackie A
Acute Lymphoproliferative Disorders
FAB L1 most common childhood leuk. best prognosis, small,homogenous, most ALLs
FAB L2 most common in adults, large blasts w/ heterogenous appearance
FAB L3 lympoblasts are uniform and large, w/ prominent nucleoli, infects B-cell line
poor prognosis. Leukemic phase of Burkitt lymphoma
Burkitt Lyphoma High-grade non-Hodgkin lymphoma phase, presents w/ abd. Mass,
t(8:14) rearrangement of MYC oncogene
Chronic Lymphoproliferative DO
Chronic Lymphocytic leukemia (CLL) in adults over 60yo. Hypercellular bm w/ abs. Lymphocytosis, (B-cell Malig.)
Hairy Cell Leukemia (HCL) massive splenomegaly w/ pancytopenia. Lymphs show hair-like projections
Prolymphocytic leukemia (PLL) >100 x10/L lymphocytosis w/ many prolymphs and thrombocytopenia
Other Lymphoid malignancies
Multiple Myeloma b-cells produce excessive IgG/IgA with decrease prod. Of other globulins.. Inc.
bld. Viscosity with M-spike on gamma region on electrophoresis. Bence-Jones
CHONS in urine.
Waldenstrom macroglobulinemia same with MM except excessive IgM with lymphadenopathy and hepato-
splenomegaly
Hodgkin Lymphoma malignant cells in lymphatic tissue. Reed-Sternberg cells(B-cell lineage)
Hon-Hodgkin Lymphoma without RS cells
Mycosis fungoides T/NK cell neoplasm. Cutaneous T-cell lymphoma; Sezary Syndrome
Acute Myeloproliferative Disorders
Acute myelogenous leukemia
FAB MO blasts exhibit myeloid markers but stain negatively
FAB M1 AML without maturation. 90% or more marrow myeloblasts
FAB M2 AML with maturation. <90% m marrow myeloblasts
FAB M3 Acute promyelocytic leuk; DIC (promyelocytes are procoagulant) w/ auer rods
FAB M4 Acute myelomonocytic leukemia
FAB M5 Acute monocytic leukemia
FAB M6 (Di Guglielmo syndrome) Acute erythroleukemia; >50 dysplastic marrow normoblasts
FAB M7 Acute megakaryocytic leuk. Blasts have cytoplasmic. blebs, w/ pancytopenia
Bilineage Leukemia 2 cell populations, myeloid and lymphoid
Biphenotypic leukemia when myeloid and lymphoid Ag are expressed on the same cell
Chronic Myeloproliferative Disorders
Chronic Myelogenous Leukemia
Essemtial Thrombocythemia
Polycythemia Vera
Chronic Idiopathic myelofibrosis
Myelodysplastic Syndromes
Refractory Anemia
Chronic Myelomonocytic Leukemia
Refractory Anemia w/ excess blasts
Hemolytic Anemia due to Extrinsic/Non-Immune Defects (normocytic, normochromic)
Microangiopathic Hemolytic Anemias
a. DIC
b. Hemolytic Uremic Syndrome
Thrombotic Thrombocytopenic Pur.
March hemoglobinuria
Hemoglobinopathies
Sickle cell Dse.
Sicke Cell Trait
Hgb C
Hgb SC
Hgb E
Hgb D
Thallasemias
Major B/Homozygo/Cooley anemia
Minor B/heterozyhous
Major A/ Hydrops fetalis
Hgb H Dse
Minor/ trait
Silent carrier
Dse. Assoc. w/ the Vascular System
Hemorrhagic Telangiectasia
Ehlers- Danlos
Hereditary Adhesion Defects
vonWillebrand Dse
Bernard-Soulier
Hereditary Aggregation & Clot Retraction
Glanzmann Thrombasthenia
Storage Pool Defects
Gray-platelet syndrome
Wiskott-Aldrich syndrome
Hermansky-Pudlak syndrome
QUANTITATIVE Platelet Disorders
Primary Thrombocytosis
Secondary (Reactive) Thrombocytosis increased plt prod. In response to thrombopoietin : <1000 x 10^9 /L
proliferation of granulocytes, Philapdelphia (t;22) chromosome
proliferaton of megakaryocytes; plt count >1000
increase in all lines esp. Red cells despite decrease erythropoietin
proliferation of erythroid, myeloid, granulocytic & megakaryocytc precursors
in bm with dyspoiesis; progressive marrow fibrosis
anemia that is not responsive to therapy
the only one that presents with leukocytosis
trilineage cytopenias common. No abs. Monocytosis
sytemic clotting due to 1) act. of coag. Cascade 2) fibrin in vessels
in children following gastrointestinal inf. ; renal damaga
def. In enzyme ADAMTS13 resp. for breakdown of vWF
forceful contact of body with hard surfaces. (sa mga players)
Valine replaces glutamic acid @ 6th position on both beta chains
Valine replaces glutamic acid @ 6th position on one beta chain
Lysine replaces glutamic acid @ 6th position on both beta chains
One sickle gene from one parent and one Hgb C gene fro another parent
Lysine replaces glutamic acid @ 26th position on beta chain
Glycine replaces glutamic acid @ 121st position on beta chains
absence of both beta chains
absence of one beta chain
absence of four alpha chains; 80% Hbg Barts(γ4), incompatible w/ life
Three alpha genes are deleted
two alpha genes deleted.
one alpha gene deleted
thin vessel walls cause mucous membrane bleeding
abn collargen prod. Causing hyperelastic skin and joint abn
lack vW factor unabling plts to adhere to collagen
lack glycoprotein Ib receptor
lack glycoprotein Iib/IIIa, the fibrinogen binding receptor; lack of throm-
basthenin/actomyosin causes clot-retraction defect
large plt, thrombocytopenia, and absenc of alpha ganules
small plts, thrombocytopenia and decreased alpha granules & dense bodies
lacks dense body granules. Px exhibits oculocutaneous albinism
uncontrolled malignant proliferation, NOT in response to thrombopoietin
>1000 x 10^9 /L