10 Hematology 2

Question 0

Acquired platelet dysfunction

Answer 0

D/t Aspirin, NSAIDs, systemic disorders (HIV, hep c)

Question 1

Causes of thrombocytopenia

Answer 1

Failure of production, increase sequestration/destruction/dilution, quinine, drugs, liver dz, DIC, pregnancy, ITP, HIV, blood transfusion, ARDS, gram neg species

Question 2

Hereditary intrinsic platelet disorder

Answer 2

Rare autosomal recessive
Glanzmann’s thrombasthenia and Bernard-Soulier syndrome

Dx: platelet aggreg test

Question 3

Idiopathic thrombocytopenia purpura

Answer 3

Autoimmune, increased platelet destruction. Children (acute and self limited) and >60 yrs (chronic)
Causes: HIV, hep c, CMV, drugs, idiopathic

Question 4

Petechaie, mucosal bleeding DDx

Answer 4

Vasculitis purpura, hemophilia, thrombocytopenia

Question 5

Thrombotic thrombocytopenic purpura

Answer 5

Can’t break down vWF…increase platelet aggregation

Classic pentad: RBC fragmentation, thrombocytopenia, fever, transient hemolytic deficits, kidney failure

Question 6

Von Willebrands disease

Answer 6

Autosomal dominant. Mild skin/mucosal bleeding, easy bruising, menorrhagia
Suspect if family hx
Definitive dx: plasma vwf antigen, vwf activity, factor 8

Question 7

Acquired causes of risk to thrombosis

Answer 7

Heparin, anti-phospholipid antibodies, hyperhomocystinemia, stasis, CHF, pregnancy, obesity, trauma, sepsis, estrogen/oral contraceptives, smoking

Question 8

Factor V Leiden mutation

Answer 8

5% prevalence in Europeans. Present in 20-60% of pts w spontaneous thrombosis

Question 9

Protein C / S deficiency

Answer 9

Venous and/or arterial embolism, warfarin induced skin necrosis
May be acquired, w liver dz, chemo, DIC, warfarin

Question 10

Hyperhomocystinemia

Answer 10

Genetic cause of risk for thromboembolism

Question 11

Disseminated intravascular coagulation

Answer 11

Excess clotting, excess bleeding.
D/t abortion, retained dead fetus, infection, malignancy, shock/burns, severe diseases
Persistent bleeding, ecchymoses, GI bleeding
Dx: D-dimer

Question 12

Hemophilias

Answer 12

Commonly factor VIII (A) or IX (B) deficient [<5% clotting factors]
Serious hemorrhage from minor injuries, hemarthrosis diagnostic, hematuria, bruising

Question 13

Amyloidosis

Answer 13

Deposition of protein in vessels, increasing vascular fragility. Perioribital purpura or purpuric rash

Question 14

Cryoglobulinemia

Answer 14

Causes small vessel vasculitis and purpura

Question 15

Hypergammaglobulinemic purpura

Answer 15

Assoc w autoimmune, SLE; vascular purpura esp lower legs

Question 16

Hyperviscosity syndrome

Answer 16

Very high IgM, seen in waldentroms macroglobulinemia

Question 17

Hereditary hemorrhagic telangiectasia

Answer 17

Arteriovenous malformations. On face, lips, oral/nasal mucosa, phalanges

Question 18

Purpura simplex

Answer 18

Common! Easy bruising dt vascular fragility

D/t deficiency in flavonoids?

Question 19

Senile purpura

Answer 19

Dark ecchymoses, forearms and hands. Skin thin and atrophic

Question 20

Idiopathic hypereosinophilic syndrome

Answer 20

Eosinophils infiltrate organs and destroy stuff.

Dx: CBC w peripheral smear, B12 levels, IgG, IgE. EKG.

Question 21

Langerhan’s Cell Histiocytosis

Answer 21

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

Question 22

Essential thrombocythemia

Answer 22

Increased platelet count, megakaryocyte hyperplasia, hemorrhagic/thrombotic tendency
Sxs: weakness, HA, parasthesias of hands/feet, mild bleeding, digital ischemia (raynauds) splenomegaly, erythromelalgia, thrombosis

Question 23

Erythromelalgia

Answer 23

Erythematous burning hands (after exposed to warm)

Question 24

Myelofibrosis

Answer 24

-->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

Question 25

Polycythemia Vera

Answer 25

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

Question 26

Other causes of erythrocytosis

Answer 26

Hypoxia, smoking, rumors, testosterone, lung dz, high altitude, diuretics, dehydration

Question 27

Acute myeloid leukemia

Answer 27

May be secondary after chemo/radiation, BM replaced w blast cells

Question 28

Acute lymphoid leukemia

Answer 28

Children <10yrs. Most common pediatric cancer. Look toxic. Fever longer than a week...red flag

Question 29

Acute leukemias

Answer 29

Ssxs: anemia, infection, easy bruising, bleeding, pallor, fatigue, fever, wt loss, bone pain May see LAD, splenomegaly, hepatomegaly

Question 30

Chronic leukemias

Answer 30

Abnormal leukocytosis w or wo cytopenia in otherwise asx person.

Question 31

Chronic lymphoid leukemia

Answer 31

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

Question 32

Chronic myeloid leukemia

Answer 32

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

Question 33

CML DDX

Answer 33

Leukemoid reactions from infection, myelofibrosis

Question 34

Myelodysplastic syndrome

Answer 34

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

Question 35

Hodgkin lymphoma

Answer 35

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

Question 36

Non-Hodgkin Lymphomas

Answer 36

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

Question 37

Big lymph nodes DDX

Answer 37

Non-Hodgkin lymphomas, infectious mono, toxoplasmosis, CMV, leukemia, sarcoidosis, lung CA, TB

Question 38

Burkitt's Lymphoma

Answer 38

Highly undiff B cell lymphoma, areas outside LNs. Mostly seen in central Africa related to EBV Most rapidly growing human tumor

Question 39

Mycosis Fungoides

Answer 39

Rare T cell tumor that affects skin, may spread to internal organs Insidious itchy plaque. Usu >50yrs

Question 40

Waldenstrom's Macroglobulinemia

Answer 40

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

Question 41

Multiple myeloma

Answer 41

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

Question 42

Primary hemochromatosis

Answer 42

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

Question 43

Secondary iron overload

Answer 43

Causes: thalassemias or sideroblastic anemias, exogenous iron, massive transfusions, liver dz