· MAIN PLAYERS OF THE HEMATOPOIETIC SYSTEM:
· EMBRYOLOGY& HISTOLOGY & PHYSIOLOGY:
· LYMPH NODES:
· SUMMARY OF HEMATOPOIETIC CELLS: BEN MET TB
· PATHOLOGY: CLASSIFICATION OF HEMATOLOGIG DISEASES: CHANT (Yes! You will be CHANT-ing how I’ve made this complex subject easier to deal with) → C-clonal hematopoietic disorders (these are your neoplastic proliferations), H-hemostasis disorders, which consist of H-hemorrhagic diathesis (aka coagulopathy), H-ereditary H-hypercoagulable states (aka hereditary thrombophilia), A-anemia, N-non-neoplastic deficiencies or proliferation of hematopoietic cell, T-transfusion reactions, T-transplant medincine.
· CLONAL HEMATOPOIETIC DISORDER: CLASSIFICATION OF CLONAL HEMATOPOIETIC DISORDER: H-hot LAMP → H-histiocytic disorders, L-lymphomas, A-acute leukemia (chronic leukemia is included in other categories), M-myeloproliferative disorders, M-myelodysplastic syndromes, M-mast cell disorders (mastocytosis), P-plasma cell dyscrasias (e.g. multiple myeloma).
· CLASSFICATION OF HISTIOCYTIC DISORDER:
· LANGERHANS CELL HISTIOCYTOSES (DENDRITIC CELLS RELATED):
· NON-LANGERHANS CELL HISTIOCYTOSES:
o CLASSIFICATION OF NON-LANGERHANS CELL HISTIOCYTOSES: H-high S-school JunioR = HS JR (patient are usually less than this age) → H-hemophagocytic lymphohistiocytosis (HLH, both familial and reactive types), S-sinus histiocytosis with massive lymphadenopathy (SHML aka Rosai-Dorfman disease), J-juvenile xanthogranuloma (JXG), R-reticulohistiocytoma
· CLASSIFICATION OF LYMPHOMA: HODGKIN’S LYMPHOMA (lymphomas containing Reed-Sternberg cells and NON-HODGKIN’S LYMPHOMA (all other lymphomas; B-cell, T-cell & NK-cell).
· CLASSIFICATION OF ACUTE LEUKEMIA: I) Acute lymphoblastic leukemias (ALL) and II) Acute myelogenous leukemias (AML)
· MYELOPROLIFERATIVE NEOPLASMS (MPN):
o CHRONIC MYELOGENOUS LEUKEMIA (CML):
o PRIMARY MYELOFIBROSIS (PMF):
o POLYCYTHEMIA RUBRA VERA (PRV):
o ESSENTIAL THROMBOCYTOSIS (ET):
· MYELODYSPLASTIC SYNDROME (aka preleukemia):
· PLASMA CELLS DYSCRASIAS:
o MGUS (MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE aka benign monoclonal gammopathy):
· MULTIPLE MYELOMA (MM aka plasma cell myeloma or as Kahler's disease)
· HEMOSTASIS DISORDERS:
· PHYSIOLOGY OF HEMOSTASIS:
· HEMOSTASIS DISORDERS: Classified into Hemorrhagic diathesis and Hereditary hypercoagulable states (thrombophilia)
· CLASSIFIED INTO I) DISORDER OF PLATELETS II) DISORDERS OF COAGULATION FACTORS
I. DISORDER OF PLATELETS: TYPES: HIT G-giant V-volleyB-ball = HIT (quantitative disorder due to ↓ platelet number) GB (qualitative disorder due to defective platelet function) → H-HIT (heparin-induced thrombocytopenia), H-HUS (hemolytic uremic syndrome), H-HELLP (H-hemolytic anemia, E-elevated L-liver enzymes, L-low P-platelets) syndrome, I-ITP (idiopathic thrombocytopenic purpura), T-TTP (thrombotic thrombocytopenic purpura), G-glanzmann’s thrombasthenia (deficiency of GpIIb/IIIa → defective platelet aGGregation), V-von Willebrand disease, B-bernard-soulier syndrome (deficiency of GpIb/IX → defective platelet adhesion).
II. DISORDERS OF COAGULATION FACTORS: Usually manifest as deeper bleeding involving the joints (hemarthrosis) and body cavities (hemoperitoneum). PT and aPTT are elevated. TYPES: Hemophilia A, Hemophilia B, and Hemophilia C (hereditary), and DIC (acquired).
· HEREDITARY HYPERCOAGULABLE STATES (aka thrombophilia): Hereditary propensity to develop thrombosis due to an abnormality in the coagulation system.
· TYPES OF HEREDITARY THROMBOPHILIA: F-feel M-my A-ass P-please = FMAP → F-FVL (20-60%), M-MTHFR mutation, A-antiphospholipid antibodies syndrome, A-antithrombin III deficiency (1-4%), P-prothrombin G20210A mutation (5-15%), P-protein C or S deficiency (total 4-10%), P-PNH-paroxymal nocturnal hemoglobinuria, P-plasminogen disorder
· ANEMIA WITH ELEVATED CRC: AHH! (imagine a bloody scream from blood loss) → A-acute blood loss, H-hemolytic anemia, H-hemophagocytic syndromes.
I. ACQUIRED CAUSES OF HEMOLYTIC ANEMIA (Often due to defects of the extraerythrocytic environment such as auto-antibodies): SIM → S-splenomegaly (e.g. from cirrhosis, CHF, amyloidosis, sarcoidosis…), I-infection (e.g. malaria, babesiosis), I-immune-mediated (autoimmune or alloimmune factors), M-microangiopathic hemolytic anemia (MAHA).
II. HEREDITARY CAUSES OF HEMOLYTIC ANEMIA (Often due to defects in the RBCs, i.e. intraerythrocytic factors): HEMoglobin = HEM → H-hemoglobin disorders, E-enzyme defect, M-membrane defects
· ANEMIA WITH REDUCED CRC: HALF, M.D. CHARMS CLITS HALF, M.D. (macrocytic anemia MCV > 100 fL); CHARMS (normocytic anemia MCV 80-100 fL); CLITS (microcytic anemia MCV < 80 fL).
o MACROCYTIC ANEMIA (MCV > 100 fL): HALF, M.D. → H-hypothyroidism, A-alcoholism, L-liver disease, F-folate &/or Vit B12 deficiency, M-myelodysplastic syndrome, D-drugs (e.g. MTX, zidovudine inhibits DNA replication).
o NORMOCYTIC ANEMIA (MCV 80-100 fL): CHARMS → C-chronic disease anemia, H-hypothyroidism, A-aplastic anemia, R-renal failure-induced anemia, M-multiple myeloma, M-myelopthisis, S-sideroblastic anemia
o MICROCYTIC ANEMIA (MCV < 80 fL): CLITS → C-chronic disease anemia, L-lead poisoning, I-iron deficiency anemia, T-thalassemia, S-sideroblastic anemia
· NON-NEOPLASTIC ELEVATION OR DEFICIENCIES OF HEMATOPOIETIC CELLS:
· ELEVATION OF HEMATOPOIETIC CELLS: Leukocytosis, thrombocytosis, and erythrocytosis (see PRV).
· NON-NEOPLASTIC DEFICIENCIES OF HEMATOPOIETIC CELLS: Leutropenia
· TYPES OF TRANSFUSION REACTION: → T-TRALI (transfusion-associated acute lung injury), H-hemolytic reaction, I-infection, I-iron overload, V-volume overload, F-febrile non-hemolytic transfusion reaction, A-anaphylactic reaction, G-GVHD (graft versus host disease).
· TRANSPLANT MEDICINE: