Hemoc Flashcards
List the factor deficiencies and what type of bleeding do they present with?
Factor 8- hemophilia A
Factor 9- hemophilia B
Factor 11- Jews
Joint bleeding, retro peritoneal bleeding, cranial bleeding
What are the three branches of coagulation disorders?
Factor deficiencies, vWD, thrombocytopenia
Which is a platelet activity problem?
VonWildebrands deficiency
List the thrombocytopenias and the type of bleeding they cause.
TTP, ITP, DIC all cause mucous membrane bleeding. Gums, petechia, purpura, vaginal bleeding
Normal platelet count?
150,000-400,000
Lab test for factor VIII deficiency and treatment?
Joint bleeding, normal PT, in erases PTT, normal thrombin time. Dx by reduced factor 8 activity, treat with factor concentrate or FFP
Describe hemophilia B.
Factor IX deficiency, x linked, bleeding into joints etc, men and less common. Normal PT, increased PTT, reduced factor 9 activity. Treat with Factor IX concentrate or FFP.
Describe the classic scenario of Factor XI deficiency and treatment.
Ashkenazi Jew with post op bleeding. Labs show prolonged PTT and reduced levels of factor XI. Treat with FFP.
What type of problem is vonWillebrands disease?
Most common severe congenital bleeding disorder. Problem with platelet adhesion not numbers. Bleeding in nasal, sinus, vaginal and GI mucosa. Labs show normal platelet count, prolonged PTT, decreased vWF. Treated with vWF concentrate and vasopressin.
What is ITP?
Immune thrombocytopenic purpura
Describe ITP.
Most common thrombocytopenia in adults. Women with other autoimmune disorder and children after a viral infection common. Presents with petechiae and purpura and gingival bleeding. NO splenomegaly.
What causes ITP?
Igg attaches to platelets and when they get to the speen they are filtered, out by macrophages. Treat with prednisone, splenectomy, IV immunoglobin
Describe Thrombotic thrombocytopenic purpura.
Seen in previously healthy young women. Consumes platelets.
What are the five classic findings in TTP?
- Thrombocytopenia
- Hemolytic anemia with schistocytes
- Fever
- Neurological signs
- Renal involvement (hematuria)
What childhood disease resembles TTP?
HUS but in children there is no neuro involvement.
How do you treat TTP?
FFP and plasma exchange
Steroids and ASA
What is DIC and what causes it?
An acquired thrombohemorrhagic disorder…. From excess thrombin and plasmin.
Something has to trigger it!
What are some signs and symptoms of DIC?
Clotting and bleeding. Ischemia, coma, respiratory failure, ulcers, inter avascular hemolysis, fever, hypotension, petechiae, purpura, hematomas, massive bleeding, epistaxis, hematuria
How do you treat DIC?
Treat the underlying cause. Heparin therapy but remember to give FFP, and platelets.
What is HIT and treatment?
Heparin induced thrombocytopenia. Less common in LMWH but it happens. Patient produced antibodies against heparin compound and they the. Become sticky and clots form. Common to have thromboembolic events. Treatment is to STOP heparin!
Acute lymphocytic leukemia (ALL)… Describe it.
Most common in children around age 4. 70% cure rate. Patient presents with anemia, bruising, infection, cns involvement, lymphadenopathy and splenomegaly.
What are the labs for ALL and the treatment plan?
Over 30% blasts! an elevated white count! anemia! need bone marrow for dx. Treat with chemo- daunorubicin, vincristine, prednisone. Methotrexate for CNS treatment.
What is CLL and how do the patients present?
Chronic lymphocytic leukemia. Cause unknown and it is men over 50 with hx of recurrent infections. Mainly B cell lymphocytes. Clinically asymptomatic with splenomegaly and lymphadenopathy. Slow indolent course.
What do the labs and treatment look like for CLL?
Lymphocytosis, SMUDGE cells, treatment is chlorambucil to reduce white count and chemo-fludarabine.
Smudge cells?
CLL
What is AML and how do the patients present?
Acute myelogenous leukemia. More common in adults with a hx of radiation or hx of chemo. Poor prognosis. Patient. Presents with fatigue, anorexia, dyspnea, lymphadenopathy, splenomegaly.
What do the labs for AML look like and what is the treatment?
Blasts >30%, Auer rods, elevated white count! and need bone marrow for dx. Treat with chemo.
Auer rods?
AML
What is CML and how does the patient present?
Chronic myelogenous leukemia. Middle aged patient with fatigue, SOB, weight loss, easy brushing and splenomegaly. Lbs are positive for Philadelphia chromosome, anemia, elevated LDH, and CBC diff shows entire white cell line. Treat with imatinib.
What is hairy cell leukemia?
Common in men 55 years with a hx of frequent infections. Patient presents with pancytopenia and splenomegaly. Hairy cells on bone smear. Treat with 2-CdA.
Describe Hodgkin’s lymphoma and how the patient presents.
Two ages….. 15-35 and over 50. Possibly from EBV. Classic Reed-Sternberg cells on smear. Patient has painless lymphadenopathy, splenomegaly, Hepatomegaly, and B symptoms (fever, night sweats, weight loss). Labs for staging.
What are the stages for Hodgkin’s and non Hodgkin’s lymphoma?
Stage 1: single lymph node
Stage 2: two or more lymph nodes on same side of diaphragm
Stage 3: lymph nodes on both sides of diaphragm
Stage 4: involvement of other organs.
Hodgkin’s lymphoma treatment?
Chemotherapy depending on stage.
Describe non Hodgkin’s lymphoma.
More common, men presents with lymphadenopathy and splenomegaly and B symptoms. Can involve skin, GI, and CNS. Staging same at Hodgkin’s. Treat with chemo.
Reed-Sternberg cells?
Hodgkin’s lymphoma.
Describe multiple myeloma.
Plasma cell dyscrasia. Bence jones proteins in the urine. Increased risk with age and blacks with risk factors of radiation or asbestos exposure.
How do patients with MM present?
Early with fatigue, weakness, weight loss. Later with bone pain, anemia, renal function changes, neuro abnormalities. Skeletal pain on exam with bruising and wasting. Recurrent infections are common.
What are classic X-ray findings with MM?
Punched out lesions in skull, ribs, spine and pelvis.
Treatment for MM?
Supportive therapy, chemotherapy melphalan, prednisone.
Bence jones proteins?
MM
Rbc x 3= hgb
Hgb x 3= hct
True
What is the normal MCV of a RBC?
80-100
List the microcytic hypo chromic anemias. There are 5.
Iron deficiency anemia Thalassemia Anemia if chronic dz Lead poisoning Sideroblastic anemia
What is the formula for a corrects retic count?
Observed retic [hct/norm hct]= corrected retic
Less than 2%= decreased production
Greater than 2% = increases destruction
List causes of iron deficiency anemia.
GI blood loss Colon cancer Vaginal blood loss Urinary blood loss Malabsorption like crones Increased demand like pregnancy Poor intake like anorexia
Lab features of iron deficiency anemia.
Microcytic, hypochromic
Decreased serum iron
Increased TBIC
Decreased iron stores in bone marrow
How to treat iron def anemia?
Elemental iron 60 mg per day
Best test to determine response to iron therapy in iron def anemia?
Retic count