Hematologic Disorders II Flashcards by Jessica Ward

What % does anemia show an ocular manifestation

10% of pts

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in what % of pts do anemia and thrombocytopenia shows ocular manifestation

40-70%

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what are some signs of ocular manifestations of anemia

conjunctival pallor ( if RBC count is 50% of normal); anemia retinopathy

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what is characteristic of Anemic retinopathy

flame shaped or dot blot hemorrhages, cotton wool spot, Roths spots

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what is an increase in the RBC mass

polycythemia

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which type of polycythemia has loss of vascular fluid

relative polycythemia

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which type of polycythemia has proliferative disease of the bone marrow ( neoplastic); absolute decrease in total rbc mass; elevated white cell and platelet counts

primary polycythemia

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which type of polycythemia has increased EPO levels caused by hypoxic conditions ( chronic heart and lung disease, oxygen chamber, and high altitude training)

secondary polycythemia

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in this condition the pt is at an elevated risk for thrombotic events; 50% increased risk of acute myeloid leukemia in 20 yrs

polycythemia

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this occurs due to a mutation at JAK2 locus in 95% of cases ( RBC hyperproliferation in bone marrow); Dx by blood test and Tx by bloodletting, AA, Jakafi

polycythemia

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these are inherited disorders affecting Hb formation

Hemoglobinopathies

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what are some inherited disorders affecting Hb formation

Sickle Cell Anemias ( SS, SC, CC, AC,AS)

Thalassemia

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this trait is common in 8% of African americans; usually asymptomatic due to one good “A” gene; confers fitness benefit against malarial infections

Sickle cell trait

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this is the most common inherited blood disorder ; normocytic- normochromic anemia; autosomal recessive; Abnormal Hb Synthesis; 70k cases in the US; more common in people of African and Hispanic Descent

Sickle Cell Anemia

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signs of this disorder include hemolytic anemia, intermittent painful crises, multiple organ damage ( spleen, GI, brain, Heart)

sickle cell anemia

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this disorder caused by a single pt mutation in the beta globulin gene; stress causes sickling ( viral illness, dehydration, hypoxia, exertion); effects are microvascular occlusion and hemolysis

sickle cell anemia

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what are the five stages of sickle cell retinopathy

1.peripheral arteriolar occlusions
2 peripheral AV anastomoses
3. sea fan neovascularization
4. vitreous hemorrhage
5. traction or rhegmatogenous retinal detachment

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which version of sickle cell is high risk for retinopathy

SC; SS is worse systemically

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this disorder is caused by absence or reduced globin chains in Hb

Thalassemias

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which type of thalassemia is caused by unstable globin chains that damage the RBC membrane > hemolysis

microcytic hypochromic anemia

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what are the two types of thalassemia

beta ( major) and alpha

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this type of thalassemia is severe, fatal in childhood, and common around the mediterranean

beta thalassemia

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this type of thalassemia is less severe; more common in S Asian and W African nations

Alpha thalassemia

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what are some causes of excessive bleeding

vitamin K def., liver disease, coagulation cascade, platelet abnormality, and meds such as Warfarin, ASA, etc

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what are lower than normal levels of platelets in the blood

thrombocytopenia

what are some causes of thrombocytopenia

pregnancy, autoimmunity, sepsis, TTP & hemolytic uremic syndrome, medications, toxins, cancer, and hypersplenism

signs of this disorder include profuse bleeding during Sx or after dental work; spontaneous bleeding from gums or nose; easy or excessive bruising; prlonged bleeding from cuts; petechiae; blood in urine or stools; heavy menstrual flows

thrombocytopenia

how do you Dx thrombocytopnia

blood draw; can be treated with transfusion/immunosuppression/splenectomy

this condition is increased risk of bleeding; X linked ; Tx with replacement therapy via infusions

Hemophilia

what are the two main types of hemophilia

Hemophilia A ( Factor VIII def) and Hemophilia B ( Factor IX def)

what are some ocular manifestations of hemophilia

hemorrhages throughout orbit, globe, and adnexa

this is the most common inherited bleeding disorder; males = femles ; 1/100 to 1/1000 affected

Von willebrand disease

what is the pathogenesis of von willebrand disease

von willebrand factor def. ( protein required for platelet adhesion)-> can be combined with factor VIII def-> poor thrombus formation due to low platelet aggregration

the effects of this disease include varying severity of coagulation def.

von willebrand disease

this is an autoimmune disorder ( primary and secondary); largely idiopathic ; pathogenesis includes inhibition of coagulation cascade regulatory proteins

Antiphospholipid syndrome

hypercoagulability is common in this disorder caused by anti-cardiolipin Ab, Anti beta 2 GP Ab, and lupus anticoagulant

Antiphospholipid syndrome

complications of this disorder include DVT, stroke, recurrent spontaneous abortion. Dx'd with blood work and Tx is blood thinners

Antiphospholipid syndrome

T or F: Antiphospholipid syndrome seen in women who have had multiple miscarriages

this is one of the most common thrombotic retinal events in hypercoagulable states

CRVO

what are some quantitative disorders of leukocyte function

leukocytosis and leukopenia

this is when you have leukoctye counts higher than normal ; pathologic; normal physiologic response to stressors

leukocytosis

this is when you have leukocyte counts lower than normal; abnormal; low wbc count predisposes to infection

leukopenia

what is a qualitative disorder

disruption of cellular function

this is uncontrolled proliferation of malignant leukocytes

leukocytosis

what is the pathogenesis of leukocytosis

single progenitor cell undergoes malignant transformation-> overcrowding of bone marrow -> decreased production and function of normal hematopoietic cells

what are the two types of leukemias

acute vs. chronic ( myeloid cells vs. Lymphoid cells)

what are some manifestations of leukemia

petechiae, purpura, and ecchymosis

signs of this disorder include anemia, infection, weight loss, bone pain, elevated uric acid, liver, spleen and lymph node enlargement and bleeding

leukemias

in this type of leukemia , you have undifferentiated or immature cells; rapid onset;

acute leukemias

which type of acute leukemia ; mean onset is 14 YO; most common childhood leukemia; 80% survival rate at 5 YO and 30-40% in adults

acute lymphoblastic leukemia

which type of acute leukemia ; most common adult leukemia ( mean age 67; treat with chemo

acute myelogenous leukemia

in this type of leukemia, mature cells do not function normally ; slow progression

chronic leukemias

this type of chronic leukemia is usuallyed Dx in adults; tx with bone marrow transplant

chronic myelogenous leukemia

this type of chronic leukemia has most Dxed over age 55; 70% asymptomatic at Dx; lymphadenopathy; suppression of humoral immunity > increased infection with encapsulated bacteria

chronic lymphocytic leukemia

T or F: Eye Signs occur in 50-70% of cases of leukemias

T ( initial presentation may related to visual complaint; ocular infiltrates assoc with a poor prognosis

what are some ophthalmoscopic findings with leukemia

retinal infiltrates ( roth Spots, cotton wool spots); ONH edema

what are some causes of lymphadenopathy

neoplastic disease, immunologic or inflammatory conditions; endocrine disorders; lipid storage disorders

what indicates lymphoproliferative disease

enlarged spleen

when we are palpating for conjunctivitis , where will we feel ?

the glands by the ears

in this condition, you get painless tumors of the lymph nodes ( can be cervical, axillary, and inguinal);

Hodgkins Lymphoma

what is the most common presenting symptom of Hodgkins Lymphoma

nontender, palpable lymphadenopathy in the neck; contiguous nodes

what are some symptoms of hodgkins

fever, night sweats, fatigue, unexplained weight loss

how is Hodgkins confirmed

by lymph node biopsy ; characterized by Reed- Sternberg cells

this condition has a higher incidence and is more common than Hodgkins; No reed Sternberg cells; more likely to affect noncontiguous lymph nodes; poorer prognosis than HL

Non Hodgkins Lymphoma

Are there ocular manifestations more in Hodgkins or Non Hodgkins

Non Hodgkins has higher incidence; but rare; 50% of 1 degree orbital malignancies ( majority of the time its in the orbit , small amt in the conjunctiva)

how are ocular manifestation of lymphoma differentially Dx'd

squamous papilloma, pyogenic granuloma, and lymphangiectasis; Dx'd via biopsy; Tx with beam radiation

T or F: pts with Sjogrens are 46 X more likely to develop Non Hodgkins lymphoma than healthy pts

T or F: 5-8% of all pts with Sjogrens Syndrome will develop non Hodgkins Lymphoma

this is the most common primary bone cancer; occurs more in elderly adults ; males more common

multiple myeloma

in this condition you get the malignant proliferation of plasma cells in bone marrow ;

multiple myeloma

what are nodular masses in bone narrow asso with multiple myeloma

plasmacytomas

what happens when you have immature hypergammaglobulinemia assoc with multiple myeloma

increased infection due to abnormal immunologlobulin production

what happens when you have increased risk of closts /ischemia leading to paraproteinemia

monoclonal gammopathy

what are some symptoms of multiple myeloma

fractures, bone pain, Hypercalcemia, anemia, recurrent infections, renal failure

how is multiple myeloma Dx'd

punched out bone lesions, monoclonal proteins in plasma or urine

how do we treat multiple myeloma

high dose chemo and allogenic bone marrow transplantation