Hematology Flashcards
Hallmarks of primary erythrocytosis (polycythemia Vera)
Facial flushing, pruritis (esp. after hot shower), splenomegaly, engorged retinal veins, headache.
*treat with phlebotomy
Organs effected in Hemachromatosis
Liver: bronze skin
Pancreas: DM
Heart: Restrictive cardiomyopathy
Endocrine: hypergonadism
S/S of iron deficiency anemia
- pagophagia (ice craving)
- pica
- angular cheilitis
- koilonychia (spooning of nails)
Diagnostic lab findings in iron deficiency anemia
- Low iron
- high TIBC
- LOW ferritin
Diagnostic lab findings in anemia of chronic disease
- low total iron
- HIGH ferritin (think all the iron is sequestered in storage)
- low TIBC
- high hepcidin
Rouleax formation on smear is associated with
Multiple myeloma
Auto agglutination on smear is associated with
Cold agglutination autoimmune hemolytic anemia
MYCOPLASMA PNEUMONIA , EBV
Howell-jolly bodies associated with
-decreases splenic function AKA sickle cell dz
Bite cells on smear associated with
Thalassemia, G6PD
Basophillic stippling is associated with
Sideroblastic anemia-lead poisoning
Auer rods are associated with
Acute myelogenous leukemia
Reed stern berg cells associated with
Hodgkin lymphoma
Pathophysiology of G6PD deficiency
Episodic, Nomocytic anemia
*G6PD protects RBC against oxidative stress, when there is a deficiency the hgb is denatured and is precipitated as HEINZ bodies
G6PD deficiency causes
1) infection
2) fava beans
3) meds: sulfa drugs, Bactrim, antimalarial
Dx of G6PD deficiency
Acute, episodic, hemolytic anemia
- bite cells
- heinz bodies
G6PD management
- self limited
- folate/iron supplementation
- blood transfusions
Management of sickle cell dz
1) IV fluids and oxygen for pain control
2) hydroxyurea
3) folic acid
Pentad of thrombotic thrombocytopenia purpura
1) thrombocytopenia (petechiae, mucocutaneous bleed)
2) neuro s/s
3) microangiopathic hemolytic anemia
4) kidney failure
5) fever
Pathophysiology of TTP
Antibodies against ADAMTS13 causes a low level. ADAMTS13 is used to cleave vWF, so with this low level there is a lot of free vWF resulting in platelet sequestration
TTP treatment
Plasmaphoresis
Hemophilia A is a deficiency in what factor?
8
Hemophilia B is a deficiency in what factor
9
*christmas dz
Hemophilia A treatment
- Factor VIII infusion
- Desmopressin (DDAVP infusion)
Howell Jolly Bodies are found in what disease and what do they signify?
Sickle cell anemia, they signify a damaged or absent spleen
What is ITP?
Idiopathic (autoimmune) throbocytopenic purpura:
-Abnormally low platelet count with normal coagulation studies for no know reason ; often ASX
- Often precipitated by a viral illness
- Autoantibody reaction against platelets
- IVIG, steroids, splenectomy is the order of treatmetnt
Target cells (codocytes) are found in what diseases?
Hemoglobinopathies such as sickle cell anemia or thalassemia
What blood disorders are hypo segmented neutrophils found in?
Macrocytic anemia, Vit. B12 & folate deficiency
Potential causes of Vit. B12 deficiency
- Pernicious anemia: lack of intrinsic factor from gastric parietal cells
- Strict Vegan diet
- Malabsorption 2/2 alcoholism
Lab findings in vitamin b 12 deficiency
- Hypersegmented neutrophils
- INC serum homocysteine, methylmalonic acid levels
- LOW b12
Clinical manifestations of sickle cell disease
1) dactylitis mC 1st presentation at 6-12 months old
2) Infections: osteomyelitis 2/2 salmonila or other encapsulated organisms, Aplastic crisis associated with parvovirus B19 infections
3) “H” shaped vertebra
4) Splenic sequestration crisis: splenomegaly, rapidly decreasing hemoglobin
5) bone pain and priapism from occlusion of schyistocytes
Hallmarks of Hodgkin’s lymphoma
- neoplasm of lymphocytes often presenting before 20 y/o or after 50 y/o
- Associated with EBV
- Painless lymphadenopathy and Systemic “B” s/s
- Involves more central lymph nodes such as neck, axiallary, mediastinal
- Reed sternberg cells on excision bx is pathopneumonic (owl’s eye appearance)
Hallmarks of Non Hodgkin Lymphoma
- Risk factors are increased age and immunosuppression
- Painless lymphadenopathy of peripheral lymph nodes with extra nodal sites common (GI, skin, CNS)
- Unpredictable course as it effects B cells, T cells, and follicular cells
- If indolent follicular form you can use Rituximab (antibody vs. CD20 on B cells) to treat
- Poor prognosis
Acute Lymphocytic Leukemia Hallmarks
- MC childhood malignancy
- Presents with Fever (MC) bone pain, CNS symptomslethargy, hepatosplenomegaly, lymphadenopathy
- Bone marrow is hypo cellular with greater than 20% blasts
- Highly response to combination chemotherapy and intrathecal methotrexate for CNS disease
Chronic lymphocytic leukemia hallmarks
- MC leukemia in adults
- B cell clonal malignancy
- Pts are often asymptomatic with fatigue, hepatosplenomegaly
- Peripheral blood smear shows SMUDGE CELLS
Acute myelogenous leukemia hallmarks
- Pancytopenia (anemia, thrombocytopenia, neutropenia ) with a leukostasis (WBC over 100,000)
- Gingival hyperplasia, bone pain
- Bone marrow shows AUER RODS
Chronic myelogenous leukemia hallmarks
- mostly asx until they develop blastic crisis with splenomegaly
- PHILIDALPHIA CHROMOSOME (Mix of 9 & 22)
- WBC over 100,000
What is polycythemia vera?
Acquired myeloproliferative disorder with overproduction of all 3 myeloid cell lines
-increased RBC, WBC, Plts
Clinical manifestations of polycythemia vera
thrombosis, pruritis, facial plethora, worse after hot bath, splenomegaly
-decreased EPO levels
Treatment of polycythemia vera
Phlebotomy
