Common Hematologic Disorders Flashcards
Hemolytic anemia
-what it is
-destruction of red blood cells, either congenital or acquired
-two types of congenital anemia + treatments
- Enzyme abnormality (G6PD deficiency) - when exposed to certain chemicals, drugs, infections, or pregnancy (avoid cause!)
- Cell membrane defect - abnormal shape causes spleen sequestering + destruction. Treatment = supportive - splenectomy, rest, O2, blood products, etc
types of acquired anemia
- acquired hemolytic
- warm-antibody
- Cold-reactive
- nutritional (iron deficiency)
- nutritional (Megaloblastic anemia)
warm-antibody anemia + treatment
-ImmunoglobulinG attachs to RBC membrane - destruction
-Steroids, immunosuppress, splenectomy + PRBC
Cold-reactive anemia + treatment
-IgM antibodies attach to red blood cells and cause agglutination (clumping) + hemolysis with exposure to cold
-treatment: avoid cold, PRBC, Plasma exchange
Megaloblastic anemia and symptoms
-B12, folate or both deficiency
-give B12 or folic acid sup
Acquired hemolytic anemia + treatment
-weird shape from various disorders - ID, drugs, chemicals, toxins in bites,
-tx: remove cause, transfuse,
symptoms of polycythemia vera + what is it
sx: HA, pruritis, coagulation disorders
what: bone marrow makes too many RBCs - blood gets thick + clot risk
mgmt polycythemia vera
-therapeutic phlebotomy + meds
-prevent hyperviscosity syndrome w/
1. hydration, but avoid volume overload
2. Move to prevent clot formation
3. Antihistamines for itching + avoid iron
what causes pruritis in polycythemia vera
-increased histamine and/or skin mast cells
Disorders of white blood cells
- Neutropenia
- Lymphoproliferative disorders
- Myeloproliferative disorders
neutropenia
-decrease in number of neutrophile cells in circulating blood
lymphoproliferative disorders
-excessive production of lymphocytes
1. CLL - chronic lymphocytic leukemia
2. ALL - acute lymphoblastic leukemia
CLL - what is it
-increase in well-differentiated lymphocytes in BMT + peripheral blood that function poorly + lead to immunodeficiency
treatment for CLL
-IVIG
-Chemo (alkylating)
-managing recurrent infections
ALL - what is it
-> 30% lymphoblasts present in bone marrow
-causes anemia, thrombocytopenia, granulocytopenia
treatment for ALL
-Chemo to eradicated leukemic cells + maintain remission
-intrathecal MTX
-potentially BMT
Myeloproliferative disorders
- CML - Chronic myelogenous leukemia
- AML - Acute myelogenous Leukemia
CML - what is it + treatment
-chromosomal abnormality resulting in lots of mature + immature granulocytes + splenomegaly
-chemo and maybe BMT
CML phases
- Chronic - can last for years, controlled with chemo
- Accelerated - difficult to treat
- Blast crisis - usually impossible to treat - couple months
AML - what it is + tx
-disorder of hematopoietic stem cells
-abnormal production of erythrocytes, neutrophils + megakaryocytes
Aplastic anemia + causes
-bone marrow mostly replaced by fat
-can be exposure to radiation, chemicals, viruses, drugs
3 bone marrow disorders
- Aplastic anemia
- Myelodysplastic syndrome
- Myelofibrosis
treatment of aplastic anemia
-PRBC
-Epo
-immunosuppressive agents
-BMT
Myelodysplastic syndrome
-idiopathic clonal hematopoietic disease
-group of disorders where blood cells are poorly formed/don’t work, resulting in suppression of bone marrow function –> fatal pancytopenia or acute leukemia
myelofibrosis
-rare blood cancer that causes scarring of bone marrow (essentially scar tissue builds up in bone marrow)
Myelofibrosis treatment
- Blood products -PRBC/plt
- Drugs: corticosteroids, EPO, interferon-alpha, hydroxyurea, thalidomide, lenalidomide
- surgery - splenectomy
- Prevention - infection prevention
How do bone marrow disorders work
-typically failure of bone marrow to produce precursor cells for peripheral hematologic components
