Blood disorders Flashcards
iron deficiency anemia
insufficient Fe
causes of iron deficiency anemia
- bleeding
- poor Fe absorption
- increased Fe demand (i.e. pregnancy)
clinical features of iron deficiency anemia
- may be asymptomatic - may present with fatigue & weakness
oral manifestations of iron deficiency anemia
- angular cheilitis - atrophic glossitis - mucosal pallor
chronic iron deficiency anemia
Plummer-Vinson syndrome
Plummer-Vinson syndrome characteristics
- rare condition of Scandinavian women - chronic iron deficiency anemia - dysphagia, atrophic mucositis of aerodigestive tracts - predisposition to oral/esophageal SCC
histological characteristics of iron deficiency anemia
hypochromatic & microcytic RBCs
treatment for iron deficiency anemia
dietary supplements of Fe (ferrous sulfate)
pernicious anemia
impaired RBC maturation due to vitamin B12 deficiency
clinical symptoms of pernicious anemia
- fatigue, weakness, headache, shortness of breath - paresthesia, tingling, numbness of extremities**
oral manifestations of pernicious anemai
- burning painful tongue, lips, buccal mucosa - loss of filiform papillae** - atrophic, pale, erythematous oral mucosa
histological characteristics of pernicious anemia
- hyperchromatic & macrocytic RBCs**
treatment of pernicious anemia
- I.M. injections vitamin B12
sickle cell anemia
inherited defect of hemoglobin, autosomal recessive, causing RBCs to be crescent “sickle” shaped
sickle cell anemia - demographic
African Americans or Mediterraneans
sickle cell anemia - clinical features
- weakness, malaise, breath shortness - infections, delayed growth
sickle cell crisis
- severe sickling - causes: hypoxia, infection, hypothermia, dehydration - symptoms: extreme pain from ischemia and infarction - long bones, lungs, abdomen most affected
radiographic features of sickle cell anemia
- “Hair-on-end” appearance of calvarium** - “Step-ladder” trabeculation of posterior mandible**
diagnosis of sickle cell anemia
electrophoesis
treatment of sickle cell anemia
supportive care: O2 and blood transfusions
thalassemia major
inherited (autosomal recessive) defect which causes decreased synthesis of alpha-globin or beta-globin Hb chains
most common form of thalassemia
thalassemia major
thalassemia major - age
early life
clinical manifestations of thalassemia major
jaundice, fever, malaise, weakness
oral manifestations of thalassemia major
- maxillary enlargement** - caused by bone marrow hyperplasia - protrusion of anterior teeth - “Chipmunk faces”
radiographic features of thalassemia major
- “hair-on-end”** - honeycombed appearance of trabeculae
RBCs appearance in thalassemia
hypochromatic & microcytic
diagnosis of thalassemia
electrophoresis
treatment of thalassemia
- blood transfusion - multiple transfusions can lead to hemochromatosis or increased Fe deposition**
leukopenia
- decreased WBC count (
agranulocytosis
- decreased granulocytes - bacterial infections common
agranulocytosis - clinical features
malaise, sore throat, fever, chills, bone pain
agranulocytosis - oral manifestations
punched-out, necrotic ulcerations of oral mucosa** and tongue
agranulocytosis - WBC count
WBC count below 1,000/mm3**
agranulocytosis - treatment
antibiotic therapy**
agranulocytosis - cause
chemotherapy** usually resolves itself if you can get rid of the cause**
cyclic neutropenia
rare idiopathic disorder, periodic decrease in neutrophils (below 3,000/mm3 for several days)
oral manifestations of cyclic neutropenia
- oral ulcerations: lips, tongue, buccal mucosa, gingiva - severe periodontal disease in children
treatment of cyclic neutropenia
antibiotic and cytokine therapy
leukocytosis
increase mature circulating WBCs
causes of leukocytosis
- normal response to infection - malignant increase in neutrophils or lymphocytes
infectious mononucleosis - cause
Epstein-Barr Virus (EBV)
infectious mononucleosis - clinical features
high fever, sore throat, *lymphadenopathy*, rashes, hepatosplenomegaly occur later
infectious mononucleosis - diagnosis
- increased WBC count (15,000 - 20,000/mm3)** - peripheral smear - monospot test
infectious mononucleosis - treatment
- symptomatic therapy: bed rest, analgesics, antipyretics, corticosteroids (rarely)
leukemia
- malignant neoplasms of transformed hematopoietic stem cells - originate in bone marrow
leukemia classifications
- acute leukemia - chronic leukemia
acute leukemia
immature “blast” cells
chronic leukemia
well differentiated WBCs
leukemia - genetic link
Philadelphia chromasome
clinical features of leukemia
- malignant proliferation of leukemic cells overwhelm bone marrow - thrombocytopenia (decreased platelets) –> bleeding, bruising
oral manifestations of leukemia
-hard/soft palate, gingival hemorrhages - candida, herpetic infections - mucosal ulcerations - gingival infiltrates (edematous gingiva)
leukemia - histology
overwhelming myeloid and/or lymphoid cells
leukemia - lab findings
- anemia - normal or elevated WBC count (above 100,000/mm3) - decreased platlets
leukemia - treatment
chemotherapy, possibly radiation, bone marrow transplant may be considered, antibiotics, transfusions
is relapse common or uncommon?
common due to chemotherapy causing second primary malignancy (complete remission is possible)
prognosis of acute leukemia
poor prognosis (children do better if treated)
prognosis of chronic leukemia
better then acute (more indolent course)
Hodgkin’s disease
malignancy arising in lymph nodes
Hodgkin’s disease - M?F
M > F
Hodgkin’s disease - age of onset
15-35 and after 50
Hodgkin’s disease - most common clinical finding
non-tender swelling
Hodgkin’s disease - histology
Reed-Sternberg (RS) cells: binucleate (“owl’s eye”) giant cells
Hodgkin’s disease - diagnosis
dependent upon histological identification of RS cells
Hodgkin’s disease - treatment
- depends on stage - can include chemotherapy and/or radiotherapy
Non-Hodgkin lymphoma
A malignant neoplasm of lymphocytes lacking RS cells
Non-Hodgkin lymphoma commonly occurs with what disease?
AIDS increased prevalence with immunosuppression & immunodeficiencies
Non-Hodgkin lymphoma - classifcation
- several systems - Kiel Classification (divided into low and high grade malignancies)
Non-Hodgkin lymphoma - M?F
M > F
Non-Hodgkin lymphoma - origin
lymph nodes (more commonly occur as extra-nodal lesions compared to Hodgkin’s lymphoma)
Non-Hodgkin lymphoma - differential diagnosis
pyogenic granuloma (difficult to differentiate clinically)
Non-Hodgkin lymphoma - radiographic features
multiple radiolucencies (helps differentiate from pyogenic granulomas)
Non-Hodgkin lymphoma - histology
- LACKS RS CELLS - looks like crushed artificat
Non-Hodgkin lymphoma - prognosis
very lethal not nearly as effective at treating as Hodgkin’s lymphoma
Burkitt’s lymphoma - associated virus
EBV
Burkitt’s lymphoma - genetic link
chromosomal translocations of chromosome 8 to chromosomes 2, 14, and 22
Burkitt’s lymphoma - classification
- endemic - non-endemic - AIDS type
Burkitt’s lymphoma - endemic demographics
- children - central Africa - head and neck region (mandible)
Burkitt’s lymphoma - non-endemic demographics
- throughout the world - more common in abdomen - slightly older (teens?)
Burkitt’s lymphoma - radiographic features
- tooth displacement, root resorption - tend to arise on gingiva and palate
Burkitt’s lymphoma - histology
- sheets of monomorphic lymphocytes - scattered macrophages with abundant cytoplasm (“starry sky”)
Burkitt’s lymphoma - treatment
high does of cyclophoshamide
multiple myeloma
malignancy of plasma cells
multiple myeloma - origin
plasma cells
multiple myeloma - age
older adults (50-60 y/o) important to know
multiple myeloma - clinical features
- renal failure - recurrent infections
multiple myeloma - oral manifestations
amyloid tongue depositions may cause macroglossia
multiple myeloma - radiographic features
punched out radiolucencies
multiple myeloma - histology
signet ring (looks like a setting in a ring)
thrombocytopenia
- decreased platelets - sequestration in spleen and liver
thrombocytopenia - clinical features
- petechiae (small blood vessel leakage) - ecchymoses, hematomas (larger vessel bleeding) - spontaneous gingival hemorrhage
thrombasthenia
- prolonged bleeding time with normal platelet counts - platelets have defective adherence or aggregate
thrombasthenia - causes
- congenital (von Willebrand’s disease) - acquired (aspirin)
von Willebrand’s disease
factor VIII disorder
vitamin C deficiency, “Scurvy”
- not common in wester countries (when seen - neglected elderly, malnourished alcoholics) - causes abnormal collagen synthesis - capillary fragility
vitamin C deficiency
- gingival inflammation, ulceration, bleeding - tooth mobility, bone loss
hereditary hemorrhagic telangiectasia
- capillary fragility - Rendu-Osler-Weber syndrome - uncommon - autosomal dominant
hereditary hemorrhagic telangiectasia - clinical features
telangiectasia (dilation of the capillaries, which causes them to appear as small red or purple clusters)
hemophilia A
- coagulation disorder - factor VIII deficiency/dysfunction - clinically indistinguishable from hemophilia B
hemophilia B
- Christmas disease - factor IX deficiency - clinically indistinguishable from hemophilia A
hemophilia - clinical features
bleeding into soft tissues, joints, muscles
hemophilia - laboratory findings
prolonged PTT
hemophilia treatmenent
clotting factor replacement therapy