10 Hematology 2 Flashcards
Acquired platelet dysfunction
D/t Aspirin, NSAIDs, systemic disorders (HIV, hep c)
Causes of thrombocytopenia
Failure of production, increase sequestration/destruction/dilution, quinine, drugs, liver dz, DIC, pregnancy, ITP, HIV, blood transfusion, ARDS, gram neg species
Hereditary intrinsic platelet disorder
Rare autosomal recessive
Glanzmann’s thrombasthenia and Bernard-Soulier syndrome
Dx: platelet aggreg test
Idiopathic thrombocytopenia purpura
Autoimmune, increased platelet destruction. Children (acute and self limited) and >60 yrs (chronic)
Causes: HIV, hep c, CMV, drugs, idiopathic
Petechaie, mucosal bleeding DDx
Vasculitis purpura, hemophilia, thrombocytopenia
Thrombotic thrombocytopenic purpura
Can’t break down vWF…increase platelet aggregation
Classic pentad: RBC fragmentation, thrombocytopenia, fever, transient hemolytic deficits, kidney failure
Von Willebrands disease
Autosomal dominant. Mild skin/mucosal bleeding, easy bruising, menorrhagia
Suspect if family hx
Definitive dx: plasma vwf antigen, vwf activity, factor 8
Acquired causes of risk to thrombosis
Heparin, anti-phospholipid antibodies, hyperhomocystinemia, stasis, CHF, pregnancy, obesity, trauma, sepsis, estrogen/oral contraceptives, smoking
Factor V Leiden mutation
5% prevalence in Europeans. Present in 20-60% of pts w spontaneous thrombosis
Protein C / S deficiency
Venous and/or arterial embolism, warfarin induced skin necrosis
May be acquired, w liver dz, chemo, DIC, warfarin
Hyperhomocystinemia
Genetic cause of risk for thromboembolism
Disseminated intravascular coagulation
Excess clotting, excess bleeding.
D/t abortion, retained dead fetus, infection, malignancy, shock/burns, severe diseases
Persistent bleeding, ecchymoses, GI bleeding
Dx: D-dimer
Hemophilias
Commonly factor VIII (A) or IX (B) deficient [<5% clotting factors]
Serious hemorrhage from minor injuries, hemarthrosis diagnostic, hematuria, bruising
Amyloidosis
Deposition of protein in vessels, increasing vascular fragility. Perioribital purpura or purpuric rash
Cryoglobulinemia
Causes small vessel vasculitis and purpura
Hypergammaglobulinemic purpura
Assoc w autoimmune, SLE; vascular purpura esp lower legs
Hyperviscosity syndrome
Very high IgM, seen in waldentroms macroglobulinemia
Hereditary hemorrhagic telangiectasia
Arteriovenous malformations. On face, lips, oral/nasal mucosa, phalanges
Purpura simplex
Common! Easy bruising dt vascular fragility
D/t deficiency in flavonoids?
Senile purpura
Dark ecchymoses, forearms and hands. Skin thin and atrophic
Idiopathic hypereosinophilic syndrome
Eosinophils infiltrate organs and destroy stuff.
Dx: CBC w peripheral smear, B12 levels, IgG, IgE. EKG.
Langerhan’s Cell Histiocytosis
Proliferative dendritic cells invade 1+organs. Eosinophilic granuloma (bone pain, swelling), Hand-Schuller-Christian dz, Letterer-Siwe dz
Dx: biopsy–Langerhan’s cells, radiograph–sharply demarcated bome lesions
Essential thrombocythemia
Increased platelet count, megakaryocyte hyperplasia, hemorrhagic/thrombotic tendency
Sxs: weakness, HA, parasthesias of hands/feet, mild bleeding, digital ischemia (raynauds) splenomegaly, erythromelalgia, thrombosis
Erythromelalgia
Erythematous burning hands (after exposed to warm)
Myelofibrosis
–>loss of hematopoietic cells, extramedullary hematopoiesis (liver, spleen)
Usu primary from neoplastic changes in marrow stem cells
Or secondary–malignancies, infections, toxins, autoimmune
Sxs: anemia, hepatomegaly/ splenomegaly, leukemia (10%), weight loss, malaise, fever
Polycythemia Vera
Chronic, myeloproliferative disorder, increase RBC mass (independent of EPO)
Sxs: increased blood vol, pruritis after warm bath, ruddy cyanosis, erythromelalgia, hepatomegaly, splenomegaly, bleeding/bruising/thrombosis
Definitive Dx: elevation of all 3 peripheral blood cells, splenomegaly, no cause for secondary erythrocytosis
Other causes of erythrocytosis
Hypoxia, smoking, rumors, testosterone, lung dz, high altitude, diuretics, dehydration
Acute myeloid leukemia
May be secondary after chemo/radiation, BM replaced w blast cells
Acute lymphoid leukemia
Children <10yrs. Most common pediatric cancer. Look toxic. Fever longer than a week…red flag
Acute leukemias
Ssxs: anemia, infection, easy bruising, bleeding, pallor, fatigue, fever, wt loss, bone pain
May see LAD, splenomegaly, hepatomegaly
Chronic leukemias
Abnormal leukocytosis w or wo cytopenia in otherwise asx person.
Chronic lymphoid leukemia
Lymphocytes accumulate in marrow then spread to lymph nodes and lymphoid tissue
Malignant transformation of B cells
Ssxs: insidious, weakness, fatigue, wt loss, fever, night sweats, hepatosplenomegaly, LAD
Hallmark: lymphocytosis peripherally and in BM
Chronic myeloid leukemia
Median age 45-55
Platelets can be high
Ssxs: fatigue, anorexia, wt loss, minor LAD, night sweats, ab fullness, bleeding problems, blast crisis where blast cell tumors develop
Dx: Philadelphia chromosome
CML DDX
Leukemoid reactions from infection, myelofibrosis
Myelodysplastic syndrome
Pre-leukemic. Somatic mutation of hematopoietic precursors
Ssxs: pallor, weakness, fatigue, fever, infxns, bleeding, exercise intolerance, angina, dizziness
Dx: peripheral smear and BM, macrocytic anemia w high RDW
Hodgkin lymphoma
Idiopathic. May be genetic, toxins, EBV, smoking, immunosuppression
*past infection w varicella, MMR, pertussis negatively assoc w development
Ssxs: painless cervical LAD, pain w alcohol, pruritis. Night sweats, fever, wt loss, nodular LNs. Pancytopenia, paraplegia, Horner’s
Dx: Reed Sternberg cells
Non-Hodgkin Lymphomas
Ssxs: peripheral LAD, anemia, result of compression of organs by big LNs
Aggressive–rapid growing LNs, fever, night sweats, wt loss, uric acid/gout
Indolent–slow growing LNs, cytopenia, hepatosplenomegaly
Dx: destruction of LN architecture and invasion of capsule, mediastinal LA on CXR
Big lymph nodes DDX
Non-Hodgkin lymphomas, infectious mono, toxoplasmosis, CMV, leukemia, sarcoidosis, lung CA, TB
Burkitt’s Lymphoma
Highly undiff B cell lymphoma, areas outside LNs. Mostly seen in central Africa related to EBV
Most rapidly growing human tumor
Mycosis Fungoides
Rare T cell tumor that affects skin, may spread to internal organs
Insidious itchy plaque. Usu >50yrs
Waldenstrom’s Macroglobulinemia
B cells –> excess IgM
Ssxs: hyperviscosity*, circ impairment, fatigue, weakness, wt loss, bleeding of skin/mucosa, visual impairment, HA, peripheral neuropathy, Raynauds, LAD, heart problems
Dx: incidentally w protein electrophoresis, elevated serum plasma viscosity
Multiple myeloma
Tumors–>excess IgG, IgA. Bence Jones proteins
Ssxs: bone pain, wt loss, fatigue, recurrent infxns, renal failure, pathological fractures, anemia
Dx: CBC, ESR, CMP, CRP. “M Spike”. X rays–punched out bone lesions
**progressive and fatal
Primary hemochromatosis
Excess iron absorption by hepcidin–>tissue damage.
Ssxs: fatigue, weakness, bronzed skin, hypergonadism, liver cirrhosis, DM
Dx: test iron, gene assay, liver bx
Tx: phlebotomy
Secondary iron overload
Causes: thalassemias or sideroblastic anemias, exogenous iron, massive transfusions, liver dz
