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28 Cards in this Set

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Platelets - Qualitative disorders are either?
acquired or congenital
Platelets - Qualitative disorders

Aquired
1. drug induced--> by far the most important of the qual disorders
*aspirin exposed platelets do not adhere but they do aggregate
2. myeloproliferative disorders
3. Uremia
Platelets - Qualitative disorders

Congenital
1. Bernard Soulier's disease
2. Glansman's disease
3. Storage pool disease
****What are the four "D's" of Quantitative Thrombocytopenia?


"The greatest thing since sliced bread"
1. Decreased production
2. Dilution (massive transfusions for example)
3. Distribution (normal amounts of platelets but are [] somewhere)
4. Increased Destruction
What are some examples of Decreased production leading to Thrombocytopenia?
aplastic anemia, congenital, drug induced, marrow infiltration, nutrional, post chemo/radiation, paroxysmal nocturnal hemoglobinuria, viral
Increased Destruction: Thrombocytopenia
-enough are produced but are being destroyed in the periph
-probably the most important of the Ds
Thrombocytopenia:
Increased Destruction Examples
-congential
-DIC
-Hemolytic Uremic Syndrome (HUS)
-Idiopathic Thrombocytopenic Purpura
-Post transfusion purpura
-Thrombotic Thromocyt purpura
Idiopathic Thrombocytopenic Purpura (ITP)
-Ab against platelet surface Ag, destroyed by immune system
-tx w/ steroids
****Thrombotic Thrombocytopenic Purpura (TTP)
Pentad of symptoms
-micro angioplastic hemolytic vascular problem--> the RBCs are being cut--> schistocytes
****Thrombocytosis

Myeloproliferative disorders
-chronic myelocytic leukemia
-Erythroblastic leukemia
-hyper-eosinophilia syndrome
-primary thrombocythemia
-myelofibrosis w/ myeloid metaplasia
-polycythemia vera--> premalig disease (too many of all cells)
***WBC disorders 1
1. acute leukemia (neoplastic WBC in the marrow
2. Chronic leukemia
3. Lymphoma (neoplastic WBC in the lymph)
4. Myelodysplastic syndromes
***Myeloid vs Lymphoid WBC disorders
1. Myeloid: from the BM
-includes precursors of granulos (neutros, basos, eosinos), monocytes, RBCs, Platelets
2. Lymph: nodes, thymus, spleen
-includes lymphos from the periph blood, lymph nodes and extranodal tissues
Acute vs Chronic WBC disorders
1. Acute: agressive clinical course (months), young malig cells predominate (blasts), "blastic"
2. Chronic:slow (years), mature malig cells predominate, "cytic"
Overview of Myeloproliferative Disorders
-disease of adults
-stem cell disorder: myeloid cell line is part of malig clone
-mature cells predominate
-*periph blood shows inc plates in ALL MPD except fibrotic stage of myelofibrosis
-BM shows hypercellularity
Myeloproliferative Disorders involving:

granulocytes, basos, eosinos
Chronic myeloid leukemia
Myeloproliferative Disorders involving:

RBCs
Polycthemia Vera
Myeloproliferative Disorders involving:

Platelets and Megakaryocytes
-Essential thrombocythemia Myelofibrosis
Myeloproliferative Disorders involving:

Monocytes
-Chronic Myelomonocytic Leukemia
Myelo-Dysplastic Syndromes - FAB classification
-myelodysplastic syndrome
-refract anemia (w/ ringed Sideroblast), (w/ excess blasts)
-chronic myelomonocytic leukemia
-refractory anemia w/ excess blasts in transformation
***WBC disorders 2
1. myeloproliferative disorders
2. Plasma cell disorders
3. other WBC disorders
****PLASMA CELL DISORDERS
-multiple myeloma
-LC disease, HC disease
-Benign monoclonal gammopathy
-Waldenstroms macroglobulinemia
-Amyloidosis
-Plasmacytoma
-Plasma cell leukemia
-Monoclonal gammopathy of undetermined significance
How to evaluate for coagulopathy?
-Hx and PE, but not enough
-CBC w/ smear
-PT (prothrombin time)
-INR (International normalized ratio)
-PTT (partial thromboplastin time)
-Platelet count
Prothrombin Time (PT)
-extrinsic pathway:
factors II, V, VII, X and fibrinogen
INR (International normalized ratio)
-ratio of pt's PT to a normal PT
PTT (partial thromboplastin time)
-factors VIII, IX, XI, XII and rarely Von Willebrand's factor
Congenital Coagulation Disorders
-Factor deficiencies (I-XII)
-Dys-fibrinogemia
-Fletcher Trait
-Fitzgerald Trait
-Von Willebrand's Disease
****Hypercoagulable states
-inherited defect of anti-clot enzymes
a. Protein C deficiency
b. Protein S deficiency
-HIT Ab
Pentad of symptoms for TPP
1. Microangioplastic hemolytic anemia
2. Fever
3. Mental Status change
4. Renal dysfunction
5. Thrombocytopenia