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Bone Marrow Failure

Clinical Medicine II

Three types of malignant neoplasms leukemia, lymphoma, multiple myeloma
Bone marrow failure aplastic anemia, myelodysplastic syndrome
Myeloproliferative conditions polycythemia vera
Tumors of hematopoietic and lymphoid tissue malignant neoplasms
4 classifications of malignant neoplasms morphologic, immunophenotypic, genetic, clinical features
Diff b/w acute and chronic leukemia acute: undifferentiated, rapid, chronic: mature cells, prolonged onset
Lymphoid cells, types leukemia, and other cancers t and b cells, ALL, CLL, lymphoma: hodgin, NHL, Multiple myeloma
Myeloid cells, types granulocytes, monocytes, AML, CML
Tumor involving lymph tissue lymphoma
Two most common types of leukemia in adults, peds AML and CLL Peds: ALL
In general what leads to leukemia chromosomal translocations→activate oncogenes and ↓Tumor Suppressor genes
What happens within the cell nl cell development arrested, rapid proliferation of tumor cells→↓apoptosis so may cells, nl cells can’t form
What are the 3 results w/I the body anemia, thrombocytopenia, neutropenia
Causes or etiology of leukemia exposures: carcinogenic, radiation, chemo,benzene, hair dyes, genetic, pesticides
What congenital d/oss predisposed for leukemia down syndrome, congenital neutropenia, fanconi anemia, neurofibromatosis
D/O of blood and blood forming organs leukemia
Fatigue, weakness doe, dizziness anemia
Infections/fever, oral throat, respiratory, uti, skin infx neutropenia
Bleeding-skin, bums, mucous membranes, GI, GU, brusing thrombocytopenia
Reasons for bone pain, LUQ pain medullary bone marrow cancer, splenomegaly
What is a painless mass lymphadenopathy
What are B symptoms generalized wt loss, fever, drenching night sweats
Signs of leukemia anemia, neutropenia, thromobycytopenia: petchiae, eccymoses, spleen, liver enlargement and lymphadenopathy
How do we Dx leukemia CBC: anemia, neutropenia, thrombocytopenia Peripheral smear: could see proliferation specific cells, dx lineage. bone marrow aspirate
Diagnosis for AML, CML, ALL, CLL auer rods, Philidelphia chromosome, CLL: smudge cells
When is CML seen middle aged
Tx in general for leukemia supportive therapy, chemo, HSCT, observation
Three stages of chemo (remission) indusction, consolidation: post remission therapy, maintenance therapy
What should we monitor for w/ chemo cytopenias: infx, bleeding, tumor lysis syndrome
Most electrolytes are elevated→renal failure tumor lysis syndrome
3 ways to use HSCT bone marrow, peripheral blood, umbilical core blood
What is autologous and allogenic mean autologous: self, allogenic: someone else: problem: graph vs. host dz
3 phases of CML initial: chronic, Accerlerated phase (transitional), blastic phase: similar to acute
What is associated w/ Philadelphia chromosome CML 9 and 22 cross over aka BCR-ABL gene
Two types of lymphoid neoplasms lymphocytic keukemia ALL, CLL and lymphoma: Hodking and Non Hodgkin lymphoma
What are proliferation of lymphoid cell lines in the bone marrar and peripheral blood lymphocytic leukemia
Wjere does lymphocytic leukemia infiltrate into lymph organs, such as lymph nodes, spleen, liver: can present w/ mas
Two types of lymphoid leukemias ALL: 85%B cell 15% T cell and CLL: B cell 90% both may/may not have a lesion
Symtoms include for ALL mild b symptoms, bone pain, HSM , LAD, CNS, mediastinal mass
Symptoms included for CLL painless LAD, B symptoms, HSM, skin
What is lymphoma a lymphoid tissue mass, two main types: Hodgkin and non Hodgkin (all others)
What are reed-sternberg cells associated with hodgkin lymphoma
What is HL associated with EBV, immunosuppression, familial link: exposures and genetics
Clinical signs of HL nontender lymphadenopthay: cervical and supraclavicular, Mediastinal mass: SOB, B sxs, pruritus, splenomegaly
Dx of HL CBC often nl, lymph node biopsy ++ for reed-sternberg cells
HL tx radiation alone, chemo alone, combined often very high survival
Main dx of HL lymph node progression in order, and reed-sternberg cells, involves B cells
Malignant solid tumors of lymph tissue can begin in any region of lymphatic sxs: Non-hodgkins lymphoma
Types of NHL small lymphocytic lymphoma, mantle cell lymphoma, burkitt lymphoma, large B;cell lymphoma, involves many types of cells
When do you biopsy a lymph node >2cm, persistence >4-6weeks, progressive increase in size
NHL presentation lymphadenopathy, HSM, B sxs, fever unknown origin
What will t-cell involvement present as skin rash and pruritis
Sxs associated w/ extranodal tissues w/ NHL pain in chest, abd, bone, GI: N/V anorexia abd fullness, CNS lethargy FND
Dx of NHL lymph node/tissue biopsy, bone marrow biopsy, morphology and phenotype, CBCs, chemistries, LFTs LDH Imaging
When do you think NHL painless masses, or abnl size etc
NHL tx radiation chemo bio therapy watchful waiting
Monoclonal abs given to carry drugs, toxins or radioactive material directly to cancer cells biology therapy
When is NHL prognosis worse >60yo, increase LDH, stage III or IV, involved extranodal dz sites >1, ↓ fxn capacity
Tumors of differentiated B cells plasma cell neoplasms, arise in bone marrow
Two types of plasma cell neoplasm PCM: single plasmacytoma, MM: multiple myeloma lesions
Causes of MM rad, benzene, herbicides, insecticides, old age,
Sxs for multiple myeloma (CRAB) calcium (elevation), renal failure, anemia, bone lesions
Why is calcium elevated extensive skeletal destruction d/t osteoclast activation →↑Ca+ in blood
Clinical signs of MM ↑Ca+ and bone pain d/t destruction of bone, can see osteoporosis, frxs, especially in spine ribs and w/movement
What is the anemia in MM bone marrow suppression, ↓ RBC d/t infiltration of tumor cells Signs: fatigue SOB, weakness
How do we dx MM w/ UA casts (sediments), and bence jones proteremia
Fragments of abs in the urine bence-jones proteiremeia
Stacked blood cells d/t excess proteins in blood Rouleaux formation
Different methods for MM dx UA, blood smear, monoclonarl proteins in serum/urine, bone marrow biopsy:clonal plasma cells, radiographs
Tests to identify MM XR for osteolytic lesions, CT, MRI
TX MM narcotics, bisphosphonates to prevent bone lesion, erythropoietin or blood trfx, abx/vaccines, metabolic
Is MM curable no,no tx: 6m death w/ tx: 2-5yrs
Causes of bone marrow failure bone marrow replacement: tumor, fibrosis, Congenital: fanconi anemia, acquired: aplastic and myelodysplasia
Diminished or absent hematopoietic precurses in the bone marrow aplastic anemia dx: pancytopenia sxs
Causes of aplastic anemia many drugs, chemicals,viruses, radiation, idiopathic
Patho of AA autoimmune, immune suppression
Clinical signs of AA infections, mucosal hemorrhage: thrombocytopenia, fatigue, cardiopulmonary compromise: anemia, pallor, but NL liver spleen lymph nodes
Dx AA pancytopenia, normocytic cells, but reduced number no abnormal cells anywhere in any bx or smears
Tx AA withdraw causative agents, supportive care, HPCT, immunosuppression
Problem w/ AA can progress to AML, MDS, and PNH
Ineffective blood cell production myelodysplastic syndrome: cytopenias, dysmorphic,
Risks of MDS >65yo, radiation, benzene chemo etc
Dx of MDS CBC: anemia, smear: could be normo, marcrocytic, but bone marrow aspirate/biopsy will be hypercelluar w/ abnl cells
Prognosis of MDS 50% 3yr, many will progress to AML will vary
Myeloproliferative d/o polycythemia vera: huge number of RBC’s
Sxs of PCV vertigo, tinnitus, HA, visual disteracnes, HTN, thrombosis, TIA splenomegaly, excoriations, GI distress (all d/t ↑RBC)
Dx of PCV CBC Erythrocytosis +/- leukocytosis, hypercellularity on bone marrow aspirate, low erythropoietin, O2 low
Tx of PCV phlebotomy and aspirin
Untreated PCV complications thrombosis, AML progression, can do well for years
Cell number and appearance/fxn of cells in AA, Myeloid, polycythemia vera AA: pancytopenia, no fxn, Myleoid: Anemia/nl Fxn: abnl PV: erythrocytosis Fxn nl
Created by: becker15
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