Term
|
Definition
Lower ph than Cellulose acetate. Run depending on aas composition. Hgb C is separated from A2 |
|
|
Term
How do you know if you have Hgb S disease opposed to trait? |
|
Definition
With disease there isn't a electrophoresis blot for A at the very end of the cellulose acetate run. |
|
|
Term
What is the genetic mutation in Hgb S Trait and which chain does it affect? |
|
Definition
Valine substituted for glutamate -->non conservative substitution (hydrophobic to philic) Beta is affected not alpha |
|
|
Term
What are findings for Hgb S trait? |
|
Definition
sickle cell crisis after exercise. Sickled cells accumulate in highly vascularized organs. Leg ulcers |
|
|
Term
What are symptoms of Hgb S disease? |
|
Definition
- chronic hemolytic anemia
- vascular obstruction by sickled cells
- splenatomegaly
|
|
|
Term
What is the major cause of death in Hgb S Disease? |
|
Definition
|
|
Term
What are test results of Hgb S disease? |
|
Definition
80% Hgb S and 20% Hgb F to compensate |
|
|
Term
What is treatment for Hgb S disease? |
|
Definition
- Inc Hgb F
- Blood Transfusion
- BM transplant with gene therapy
|
|
|
Term
What is the cause of Hgb C disease? |
|
Definition
Lysine is substituted for gutamate on beta chain (conservative subsitution) |
|
|
Term
What are tests for Hgb SC disease? |
|
Definition
- target cells
- ****crystals poke out of cell
- less anemia than Hgb SS
|
|
|
Term
What are some features of Plasmodium Falciparum and where do you get it from? |
|
Definition
--Anopheles Mosquitoes in tropical regions - banana shaped gametocytes
- Rings form everywhere
|
|
|
Term
What are results of Plasmodium Falciparum infection? |
|
Definition
- Massive Hemolysis
- Cerebral Malaria--->causes death
- fevers
|
|
|
Term
What are the results of Plasmodium Vivax and where are people effected? |
|
Definition
- dots
- enter through Duffy antigen
- chronic bc they stay in liver
- in US not Africa
|
|
|
Term
What is a gentler version of falciparum? |
|
Definition
|
|
Term
What is a gentlier version of vivax? |
|
Definition
|
|
Term
Which parasite is spread through ticks and what does it cause? |
|
Definition
Babesiosis - to immunocompromised
- can be with Lyme disease
- or spread through transfusion
|
|
|
Term
What is the normal ratio of marrow to fat in the BM? What is hypercellular? Hypocellular? |
|
Definition
1:1 or 2:1 Hypercellular is >4:1 Hypocellular is <1:4 |
|
|
Term
What is the normal myeloid: erythroid ratio in the BM and why? |
|
Definition
2:1, bc WBCs live shorter t1/2, and RBCs accumulate, so WBCs need to be made more often |
|
|
Term
What are some causes of hypoplasia? |
|
Definition
- old age (bm is less active)
- Aplastic anemia
- Reticular fibrosis
|
|
|
Term
What is the difference bw a myelocyte and a metamyelocyte? |
|
Definition
Myelocyte has a round nucleus and a metamyelocyte has an indented nucleus |
|
|
Term
What cells does the Acid Phosphatase stain? |
|
Definition
|
|
Term
What stain will help you tell the difference bw an infection and a leukemia? |
|
Definition
Leukocyte alkaline phosphatase score infection: high score leukemia: low score |
|
|
Term
What is the PAS-carbohydrate stain used for? |
|
Definition
Distinguishes Gaucher's from Neiman Pick (lymphoid). Erythroid Leukemia |
|
|
Term
What is Chediak-Higashi Syndrome caused by and what in the prognosis? |
|
Definition
Giant fused granules in Neutrophils and lymphs - Cells engulf but do not kill microbes
- die as child
- skin hypopigmentation
|
|
|
Term
What are the Different types of Myelodysplastic Disorders? |
|
Definition
|
|
Term
What do you get Myeloproliferative Disorders and name the different kinds? |
|
Definition
when Cell cycle checkpoints don't work - Chronic Myeloid Leukemia
- Chronic Idiopathic Myelofibrosis
- **Polycythemia Vera
- Essential Thrombocythemia
|
|
|
Term
What mutation is usually present in Chronic Myeloid Leukemia? |
|
Definition
Philadelphia Chromosome (bcr-abl fusion)-Tyr Kinase always on |
|
|
Term
What is the presentation of someone with Chronic Myeloid Leukemia? |
|
Definition
- Middle Aged
- elevated WBC
- fever, weakness, bleeding,splenomegaly
- tender lower sternum
- no lymphadenophathy
- bruises
|
|
|
Term
What are lab findings for Chronic Idiopathic Myelofibrosis? |
|
Definition
looks strange bc cells are make in the worng places - Dacrocytes (tear drop)
- Aniso/poikilo, Basophilic Stipping
- <5% blasts
- Elevated LAP
|
|
|
Term
What are characteristics of Polycythemia Vera? |
|
Definition
Panmyelosis in BM (100% cellularity) Splenomegaly - cells are very sensitive to EPO
|
|
|
Term
What is therapy and prognosis for Polycythemia Vera? |
|
Definition
- Bloodletting to dec Hct
- stable
- BM fibrosis, splenomegaly, teardrop
|
|
|
Term
What is the therapy and Prognosis of Essential Thrombocythemia? |
|
Definition
- long after diagnosis
- Plts can clog vessels
- txt: suppress plt production through radiation and drugs --->increases risk of leukemic transformation
|
|
|
Term
What are the demographic and peripheral features of Myelodysplastic Syndromes? |
|
Definition
- Older adults
- ***Oval macrocytes (norm B12)
- Dimorphic RBC
- Poikilocytosis from irregular shape
- Basophilic Stippling, Howell-Jolly Bodies, Siderocytes
- abnormal plt
|
|
|
Term
What are BM changes of Myelodysplastic Syndromes? |
|
Definition
Since Hematopoetic SCs are messed up: - RBC with multiple nuclei and abnormal shapes (lobes)
- Nuclear Bridging from trying to divide
- Megaloblastoid changes
- Basophilia, 2nd granules absent
|
|
|
Term
What is 5q-Syndrome, demographic, characteristics, and treatment? |
|
Definition
Deletion of q arm of chromosome 5 - Women >50
- Refractory macrocytic anemia
- thrombocytosis
- erythroid hypoplasia
Lenalidomide-forces precursor cells to develop |
|
|
Term
What is the treatment for Myelodysplastic syndromes? |
|
Definition
Palliative (bc we don't understand it): transfusions, colonly stimulating factor (to inc cycling of progenator cells) |
|
|
Term
What is the only cure for myelodysplastic syndromes? |
|
Definition
Replace Hematopoetic progenitors: - BM transplant, Cord blood, Low Intensity Transplant
|
|
|
Term
What is the presentation and demographics of someone Acute promyelocytic leukemia? |
|
Definition
- females, adults
- SUDDEN AND SEVERE progression
- bleeding; clot and fibrinolysis everywhere-
give Heparin to present coag cascade |
|
|
Term
What is the staininglike with Acute Promyelocytic Leukemia? |
|
Definition
+ Myeloperoxidase + Chloroacetate (specific for Neut) - Nonspecific Esterase |
|
|
Term
WHat are characteristics of AML from Alkylating agents and radiation therapy and what is the prognosis? |
|
Definition
- 5 yr after txt
- MDS-->all myeloid lines affected
- Ringed Sideroblasts
- >20%blasts
- poor prognosis
|
|
|
Term
What is the presentation of a pt with AML with Acute Myelomonocytic Leukemia? |
|
Definition
- 25% of AML cases
- myeloid and monocytic cells
- high WBC
- thrombocytopenia
- >30% blasts in BM: 20% non-rbc are monocytic
- ***elevated serum Muramidase
|
|
|
Term
What is the AML kids get? |
|
Definition
Acute monoblastic Leukemia |
|
|
Term
What is the presentation of AML with Acute Monoblastic Leukemia? |
|
Definition
- kids
- weakness/bleeding
- skin rash
- high muramidase (from monocytes)
- gums overgrown
- DIC if monocytes lyse
|
|
|
Term
What is Hodgkin's Lymphoma, it's prelavence, and demographics? |
|
Definition
- in Children
- proliferation of: lymphocytes, B-cells, eos, Reed-Sternberg cells (dinucleated/owl cells)
- 30% of lymphomas
- usually in one node; cells don't mature proper
|
|
|
Term
What are immunotyping results of Chronic Lymphocytic Leukemia? |
|
Definition
- B-cell markers (CD--) > 10
- Weak surface Ig with light chain restriction
|
|
|
Term
What does the peripheral smear look like with Chronic Lymphocytic Leukemia? |
|
Definition
- lots of small lymphocytes
- smudge cells (from breaking of fragile lymphocytes)
|
|
|
Term
What does the BM look like in a pt with Chronic Lymphocytic Leukemia? |
|
Definition
- no fat
- no Erythroid progenators or Monocytes-->anemia and bleeding
- immature cells, huge lymphoblasts
- lypho with cleaved nucleus
|
|
|
Term
What distinguishes Hairy Cell Leukemia; cells and tests? |
|
Definition
- Splenomegaly
- dry tap
- + TRAP
- + for IG
- hairy cells
- mature lymph in BM
|
|
|
Term
What is Waldenstrom Macroglobulinemia and why isn't it a leukemia? Also, how bad is it? |
|
Definition
B-cell Neoplasm: - IgM: plasma cells won't stop making antibody
- Small lymphs and plasma cells take over node
- not too bad, incurable
- not enough blasts to be leukemia
- Roullexing
|
|
|
Term
What is Multiple Myeloma and who does it affect? |
|
Definition
- Malignant Proliferation of plasma cells
- takes Ca2+ out of bone causing fractures
- light chain Bence Jones protein in urine
- from tumor burken
- 63yoa
|
|
|
Term
What is Burkitt's Lymphoma ass with, and describe demographics, characteristics? |
|
Definition
B-cell disease with high malignancy - kids, AIDs PT, surpressed immune system
- large jaw
- EBV
- cells regrese to immature
|
|
|
Term
What is Sezary Cell Syndrome, it's immunotyping, etc? |
|
Definition
T-cell disease from skin tumors - CDs <10
- cleaved nucleus
- poor prognosis if other organs are involved
|
|
|
Term
What are symptoms of ALL at presentation? |
|
Definition
- lymphoblasts in CSF -need chemo that cross BBB
- fatigue (no RBC), fever, bleeding
- enlarged Lymph nodes
- Hepatosplenomegaly
- bone pain if stuffed
- Blasts in ovaries/testes
|
|
|
Term
What are the 3 morphologies of Blasts with ALL? |
|
Definition
- L1-small blasts: 2x cytes, flat round nucleus
- L2-Larger than L1: irregularly shaped nucleus
- L3-Large Blasts: Round/oval nuclei, lots of blue cytoplasm w/vacuoles
|
|
|
Term
What is Immature B-Cell ALL, it's prevalence, and prognosis as an ALL? |
|
Definition
- most common
- immunoglobulin is ABSENT on surface and cyto
- Best prognosis bc cells aren't active
|
|
|
Term
What is B-Cell ALL and prognosis? |
|
Definition
- Surface Ig with Lambda or Kappa Light Chain expressed
- worst prognosis
|
|
|
Term
What is T-Cell ALL demographics, characteristics, prognosis? |
|
Definition
- CD<10 are expressed Tdt+
- Teenage boys
- mass in chest
- inc blood blasts
- cells in CSF
- infiltration of other sites
- Aggressive Therapy improves prognosis
|
|
|