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Hematology
Midterm 4
57
Biology
Undergraduate 4
11/08/2008

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Cards

Term
What is the Citrate gel?
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
bacterial infections
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
Plasmodium malariae
Term
What is a gentlier version of vivax?
Definition
Plasmodiu Ovale
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
T-cells
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
RA, RARS, RAEB, CML
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
  1. L1-small blasts: 2x cytes, flat round nucleus
  2. L2-Larger than L1: irregularly shaped nucleus
  3. 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
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