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Causes of Microcytosis-low MCV

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1) Iron definiency 2) Thalassemia 3) Sideroblastic anemia 4) Anemia of chronic disease

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Only microcytic anemia with elevated reticulocyte count

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alpha thalassemia with 3 genes deleted has elevated reticulocyte count

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Type of anemia that can be micro- or macrocytic

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Sideroblastic

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Causes of Macrocytic anemia-high MCV

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1) B12 deficiency 2) folate deficiency 3) Sideroblastic anemia 4) Alcoholism 5) Antimetab. Rxs- azathioprine, 6-mercaptopurine or hydroxyurea 6) liver disease 7) hypothyroidism 8) zidovudine, phenytoin 9) myelodysplastic syndrome

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Causes of Normocytic anemia

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acute blood loss or hemolysis -no time for MCV to change, eventually hemolysis will increase retic. count and MCV will increase.

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Tx for anemia

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packed RBCs -Hct 25-30 in elderly or heart disease -young and asxs will make own RBCs, no need for tx

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Packed RBCs

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-Concentrated blood. -Each unit should raise Hct 3 points.

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Fresh Frozen Plasma

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Plasma and clotting factors -provides clotting factors in those with elevated PT, aPTT or INR and bleeding

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Treatment for Hemophilia A, B and vWF deficiency

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1) recombinant factor 2) desmopressin -FFP does not work, factors are under endothelial cells, need to be released.

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Cryoprecipitate

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-higher concentration of clotting factors -replaces fibrinogen-->Tx for DIC -never used first

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Best test for microcytic anemia

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Iron studies

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Iron study association: Low Ferritin: High Iron: Normal Iron:

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Low Ferritin-->iron deficiency. Increase TIBC High iron--> Sideroblastic anemia. Decrease TIBC Normal iron-->Thalassemia

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Iron deficiency unique labs

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RDW increased Elevated platelet count most accurate test bone marrow biopsy with stain for iron

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Sideroblastic anemia unique labs

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Most accurate-Prussian blue for ringed sideroblasts basophilic stippling

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Thalassemia unique labs

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most accurate- electrophoresis alpha thal-genetic studies

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B12 def. caused by:

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1) Pernicious anemia -auto-Abs 2) Pancreatic insufficiency 3) dietary deficiency (vegan/veg) 4) Crohn or disease 5) blind loop syndrome (gastrectomy or gastric by pass for wt loss) 6) Diphyllobothrium latum (tape worm) 7) Celiac disease (def all 3)

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Folate def. caused by:

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1) dietary deficiency (goat's milk no folate, lim. B12 and iron) 2) Psioriassi and skin loss or turnover 3) Rxs: phenytoin, sulfas, methotrexate with RA

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B12 Sxs

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Neuro abnorm- MC- peripheral neuropathy, post column damage to position and vibration or "subacute combined" -ataxia -dementia (rare)

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Increase in methylmalonic acid seen in:

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B12 deficiency only -all other tests and smear are same in folate and B12

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Pernicious anemia confirmed with:

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anti-intrinsic factor and anti-parietal cell Abs

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Sickle Cell common manifestations

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1) increase retic count 2) Bilirubin gallstones 3) increase infxns from autosplenectomy, esp. encapsulated organisms 4) Osteomyelitis, Salmonella 5) Retinopathy 6) Stroke 7) Enlarged heart with hyperdynamic features and systolic murmur 8) LE ulcers 9) Avascular necrosis of femoral head

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Dx Sickle Cell

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best initial- peripheral smear most accurate- Hb electrophoresis

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Basophilic stippling

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Lead poisoning

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Howell-Jolly bodies

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Sickle cell

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Bite cells

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G-6-PD

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Schistocytes

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intravascular hemolysis

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Morulae

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Ehrlichia infxn (seen in neutrophils)

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Sickle Cell Tx

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1) O2/hydration/analgesia 2) fever or white cell count--> Abx 3) Folic acid chronic basis 4) pneumococcal vaccination 5) Hydroxyurea prevents recurrences of crisis by increasing HbF (3-4 crises/yr give)

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Very Severe Sickle Cell Tx

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exchange transfusion -do if have... acute chest syndrome, priapism, stroke, visual disturbance from retinal infarct

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Only manifestation of sickle cell trait:

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inability to concentrate urine No Tx

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Splenomegaly, intermittent jaundice, episodes of jaundice, FHx anemia or hemolysis, bilirubin gallstones

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Hereditary spherocytosis

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Spherocytosis Dx Tests

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-low MCV -increased MCHC -Negative Coombs test most accurate test- osmotic fragility

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Spherocytosis Tx

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Chronic folic acid replacement Splenectomy

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Autoimmune (Warm or IgG) Hemolysis Most Accurate Test

answer

Coombs test- detects IgG AB on surface of RBCs -indirect a/w greater amt of Abs--detect same thing

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Autoimmune Hemolysis Tx

answer

1) best initial-prednisone 2) recurrent-splenectomy 3) severe, acute not responding to splenectomy--> IVIG 4) if splenectomy doesn't control hemolysis-Rituximab 5) Other Txs: cyclophosphomide, Cyclosporine, Azathioprine, mycophenolate mofetil

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Cold Agglutinin Dx a/w

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EB virus, Waldenstrom macroglobulinemia, or mycoplasma

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numbness or mottling of nose, ears, fingers, and toes. Resolve when warmed.

answer

Cold Agglutinin Dx

question

Cold Agglutinin Dx Test and Tx

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Dx Test- direct Coombs + for complement only. Normal smear, may show spherocytes Tx-stay warm, Rituximab, Cyclophosphamide, Cyclosporine, or other immunosuppressives

question

Cryoglobulins a/w

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1) Hep C 2) Joint pain 3) Glomerulonephritis

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G6PD stressors

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-infxn -dapsone, quinidine, sulfa, quinidine, nitrofurantoin -fava beans

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G6PD Dx Test

answer

best initial-bite cells and Heinz bodies most accurate-G6PD level 1-2 months after hemolytic episode.

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sudden anemia, jaundice, normal spleen w/infxn or on new drug. AA or Mediterranean man

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G6PD -X-linked rec

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Intravascular hemolysis with schistocytes (frag cells), thrombocytopenia, renal insuff.

answer

HUS or TTP -normal PT/aPTT and neg. Coombs

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HUS a/w

answer

E. coli O157:H7 in children

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TTP a/w

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ticlopidine, clopidogrel, cyclopsorine, and AIDS Sxs- neurologic dxs (szrs/confusion), fevers, and adults.

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HUS and TTP Tx

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plasmapheresis or plasma exchange in severe cases -Never give platelets

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Paroxysmal Nocturnal Hemoglobinuria what is it?

answer

clonal stem cell defect with increased sensitivity of RBCs to Complement in acidosis, which occurs at night as respirations decrease and pH drops slightly. -decay accelerating factor- CD55 and CD59 -overactivation of complement leads to hemolysis and thrombosis

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PNH Dx Test

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CBC-pancytopenia and anemia most accurate- decrease CD55 and CD59 wrong answer-flow cytometry, Ham test or sucrose test if CD59/55 are in answer.

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PNH Tx

answer

1) best initial-prednisone 2) cure- bone marrow transplant 3) Ecluzimab-inactivates C5, decrease C` activation 4) supportive care- folic acid and transfusions if needed.

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Aplastic Anemia

answer

Pancytopenia of unclear origin -any infxn, cancer can invade BM and decrease production -most accurate test-BM biopsy

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Aplastic Anemia Tx

answer

Rxs-cyclosporine-inhibit T-cells Cure- allogeneic bone marrow transplant (50 or no BMT match- antithymocyte globulin and cyclopsorine, alt. tacrolimus

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Polycythemia vera definition

answer

-unregulated overproduction of all 3 cell lines. -JAK2 mutation. -RBCs grow wild, low Epo.

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Sxs: -Headache, blurred vision, tinnitus -HTN -Fatigue -Splenomegaly -Thrombosis -Engorged blood vessels -Pruritus, esp after shower

answer

PCV

question

PCV most accurate test Other test Hct- RBC- Basophils- O2- Epo- B12- MCV

answer

most accurate- JAK2 mutation Hct- >60% RBC- increased O2- normal Epo- low B12- elevated MV-low

question

PCV Tx

answer

1) Phlebotomy 2) Hydroxyurea

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Essential Thyombocytosis definition

answer

"platelet cancer" markedly elevated platelet count above 1 million-->thrombosis and bleeding

question

Essential Thrombocytosis Tx

answer

<60 yrs, asxs, and platelet count 60, thromboses or platelet count >1.5 million=hydroxyurea best initial tx.

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Myelofibrosis definition

answer

"bone marrow fibrosis" -get pancytopenia with shift of blood production to liver and spleen-->both become enlarged

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Myelofibrosis Dx and Tx

answer

-tear drop cells on blood smear -Tx-thalidomide and lenalidomide--TNF inhibitors that increase BM production. -BMT occassionally done in patients <55

question

Acute Leukemia Sxs

answer

-fatigue -infection -bleeding -pancytopenia with normal or elevated WBC

question

Acute Leukemia Tests -Best initial -Most accurate -Best indicator of prognosis Other keys

answer

-Best initial-blood smear showing blasts -Most accurate-flow cytometry -Best indicator of prognosis-cytogenetics assesses chromosomal characteristics/type M3 or preomyelocytic leukemia MC a/w Auer rods. M3-->DIC

question

Add what to Tx of M3

answer

all-trans-retinoic-acid (ATRA)

question

Add what to ALL Tx

answer

intrathecal chemotherapy ex: MTX

question

Sxs: -pruritus -splenomegaly -fatigue, night sweats, fever -persistent high WBC that is all neutrophils

answer

CML BCR-ABL 9:22 Philly chromosome translocation

question

CML DX tests best first most accurate

answer

Best first test- LAP (leukocyte alkaline phosphatase score) -LAP is low in CML -Most accurate test/LAP not listed- BCR-ABL via PCR -blasts <5%

question

CML Tx-

answer

best initial tx-tyrosine kinase inhibitors -imatinib, dasatinib, nilotinib Cure-BMT

question

CLL Tx- Early/Asxs- Rxs- Other-

answer

Early/Asxs-observe Rxs-Chlorambucil or fludarabine w/Rituximab Other- Corticosteroids, IVIG -radiotherapy

question

Hodgkin Lymphoma Stage I-II: Stage III-IV Relapse

answer

Stage I-II: ABVD, then radiation Stage III-IV-ABVD w/radiation Relapse- high dose chemo-->BMT

question

ABVD

answer

Adriamycin-->cardiotoxic Bleomycin-->lung fibrosis Vinblastine Dacarbazine

question

CHOP-R

answer

Cyclophosphamide Hydroxydoxorubicin (Adriamycin) Oncovin (Vincristine) Prednisone Rituximab

question

B cell NHL

answer

Burkitt lympphoma ++ aggressive diffuse large B cell lymphoma + aggressive Follicular lymphoma ~ Mantle cell lymphoma ~

question

T cell NHL

answer

Mycosis fungoides Anaplastic large cell lymphoma

question

Rx Tx for NHL

answer

CHOP-R, then radiation MTX- Px CNS Relapse--> BMT

question

Tx Multiple Myeloma

answer

65 Melphalan, Bortezomib, prednisone Tx Sxs- kidney, bones, anemia too

question

Tx Waldenstrom's Macroglobulinemia

answer

Chlorambucil, Fludarabine, Thalidomide, Rituximab -Corticosteroids -plasmapheresis to decrease IgM

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