Hematologic Disorders II Flashcards
What % does anemia show an ocular manifestation
10% of pts
in what % of pts do anemia and thrombocytopenia shows ocular manifestation
40-70%
what are some signs of ocular manifestations of anemia
conjunctival pallor ( if RBC count is 50% of normal); anemia retinopathy
what is characteristic of Anemic retinopathy
flame shaped or dot blot hemorrhages, cotton wool spot, Roths spots
what is an increase in the RBC mass
polycythemia
which type of polycythemia has loss of vascular fluid
relative polycythemia
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
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
in this condition the pt is at an elevated risk for thrombotic events; 50% increased risk of acute myeloid leukemia in 20 yrs
polycythemia
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
these are inherited disorders affecting Hb formation
Hemoglobinopathies
what are some inherited disorders affecting Hb formation
Sickle Cell Anemias ( SS, SC, CC, AC,AS)
Thalassemia
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
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
signs of this disorder include hemolytic anemia, intermittent painful crises, multiple organ damage ( spleen, GI, brain, Heart)
sickle cell anemia
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
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
which version of sickle cell is high risk for retinopathy
SC; SS is worse systemically
this disorder is caused by absence or reduced globin chains in Hb
Thalassemias
which type of thalassemia is caused by unstable globin chains that damage the RBC membrane > hemolysis
microcytic hypochromic anemia
what are the two types of thalassemia
beta ( major) and alpha
this type of thalassemia is severe, fatal in childhood, and common around the mediterranean
beta thalassemia
this type of thalassemia is less severe; more common in S Asian and W African nations
Alpha thalassemia
what are some causes of excessive bleeding
vitamin K def., liver disease, coagulation cascade, platelet abnormality, and meds such as Warfarin, ASA, etc
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
T
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
T or F: 5-8% of all pts with Sjogrens Syndrome will develop non Hodgkins Lymphoma
T
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
