Cytopenia and Cythemia Flashcards
What type of anemia is marked by decreased reticuloctes, hypocellular marrow, which increased erythropoietin, normal MCV, and normal haptoglobin?
Aplastic anemia - characterized by pancytopenia and a hypocellular bone marrow filled with fat and stroma
Causes of aplastic anemia
Often due to destruction of multipotent hematopoietic stem cells:
- Radiation
- Drugs (i.e. chloramphenicol)
- Chemicals (benzene and glue sniffing)
- Infections (especially hepatitis C)
Pancytopenia
- Anemia
- Agranulocytosis
- Thrombocytopenia
Signs of pancytopenia
- Fatigue (due to decreased RBCs)
- Infections (due to decreased WBCs)
- Bleeding (decreased platelets)
Pancytopenia w/ decreased reticulocytes
Consistent w/ aplastic anemia
Cyclic neutropenia
Disease characterized by accelerated apoptosis of neutrophil precursors
Nuclear fragmentation and membrane blebbing are features seen in apoptosis
Inherited aplastic anemia caused by abnormal DNA interstrand crosslink repair
Fanconi anemia
Signs of Fanconi anemia
Hypoplasia of kidneys, thumbs, spleen, and radii
Most common cause of pure red cell aplasia
Immune-mediated loss of erythroid precursor cells
PRCA - normal WBCs and platelets
Disease associated w/ thymoma (mediastinal tumor), myasthenia gravis, and parvovirus B19 infection
Pure red cell aplasia
What type of infection is characterized by intranuclear inclusions w/i pronormoblasts
Parvovirus B19 infection - cause of pure red cell aplasia in individuals w/ chronic hemolytic anemia
Most common viral cause of aplastic crisis in Pt’s w/ sickle cell and other chronic hemolytic disorders?
Parvovirus B19 infection of erythroid progenitor cells (pronormoblasts)
What type of virus is parvo B19?
Nonenveloped ssDNA virus
Tick-borne infection characterized by flu-like and neurologic symptoms along with leukopenia and asterisk-shaped cytoplasmic inclusions in neutrophils and monocytes.
Ehrlichia chaffeensis
What vector transmits Ehrlichia chaffeensis
Amblyomma americanum (lone star tick)
A significant leukocytosis in the setting of a bacterial infection with an increased leukocyte alkaline phosphatase score
Consistent w/ leukmoid reaction
Basophilic oval inclusions in neutrophils (Dohle bodies) along with increased LAP (leukocyte alkaline phosphatase) and peripheral leukocytosis (white cell count over 50,000).
Leukmoid reaction
Neutrophilia w/o left shift
May result from demargination of neutrophils in the peripheral blood - can be caused by corticosteroids
Signs of infectious mononucleosis
- Sore throat
- Fever
- Palatal petechiae
- Lymphadenopathy
- Splenomegaly
- Atypical lymphocytsosis in peripheral blood smear
Atypical lymphocytes in mono
Larger than normal w/ abundant cytoplasm, the periphery of which may be condensed and more blue in appearance
How long should contact sports be avoided in a Pt w/ infectious mono?
4 wks
EBv infects what cells?
CD21+ B lymphocytes
EBv cuases proliferation of what cells?
CD8+ T lymphocytes
Seen as atypical lymphocyte in peripheral smear and paracortex of nodes
Heterophile Ab test
Monospot test
Metastatic disease associated w/ EBv
Burkitt lymphoma
Nasopharyngeal carcinoma
Hodgkin lymphoma
Diffuse large B cell lymphoma
Primary CNS lymphoma
Heterophile-negative mononucleosis causes
Cytomegalovirus (second most common cause of mononucleosis)
Neutrophil presence in lymph nodes
May be present w/ acute bacterial lymphadenitis
Neutrophils do not typically preside in lymph nodes
Medullary sinuses of lymph nodes may be filled w/ macrophages under what conditions?
Crhonic inflammatory disorders - i.e. TB
Cat-scratch fever infectious organism
Bartonella henselae
Bartonella henselae causes what disease(s)
Cat-scratch disease and bacillary angiomastosis
Infectious disease of immunosuppresed individuals characterized by vascular lesions of skin
Bacillary angiomatosis - caused by Bartonella henselae
Transmission of Bartonella henseale
Pathogen is typically transmitted from the feces of cat fleas via cat sratch
Lymph node biopsy showing necrotizing granulomas and stellate-shaped microabscesses
Characteristic of cat scratch fever
Relative polycythemia
Polycythemia w/ normal RBC mass
Causes of relative polycythemia
Dehydration
Excessive diuresis
Absolute polycythemia
Polycythemia w/ increased RBC mass
Causes of absolute polycythemia
Primary polycythemia (w/ decreased EPO levels)
Secondary polythemia (increased EPO levels)
What stimulates interstitial cells in kidney to increase release of EPO?
Anemia-induced tissue hypoxia
Normal bone marrow cellularity
~50% (decreases w/ age over 50)
Paroxysmal nocturnal hemoglobinuria presdisposes Pt’s to what type of anemia?
Aplastic anemia
Most cases of Fanconi anemia develop what?
Acute myelogenous leukemia
What promotes proliferation and differentiation of progenitor cells of neutrophils, eosinophils, and monocytes/macrophages in the bone marrow?
Granulocyte-macrophage colony-stimulating factor (GM-CSF)
Recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF) can be used to treat?
Neutropenia due to stem cell destruction
Myeloophthisic anemia
Results from space-occupying lesions of the bone marrow (ophthisic means hard)
Such as metastases (prostate cancer in men, breast cancer in women), granulomas (TB or sarcoidosie) or fibrosis (myelofibrosis)
Presence of leukoerythroblastosis in the peripheral blood and the presence of teardrop RBCs characterizes?
Myelophthisic anemia -
leukoerythroblastosis refers to finding in the peripheral blood
immature white cells, such as myelocytes and metamyelocytes, and immature red blood cells, such as nucleated red blood cells (nRBCs).
Increased destruction of neutrophils causes what marrow finding?
Hyperplasia of the bone marrow with and increase in the number of granulocytic precursors
Causes of increased neutrophil destruction
- Immune mediated (Felty’s syndrome)
- Drug rx’ns (aminopyrine and some sulfonamides)
- Sequestration in the spleen due to hypersplenism
- Increased utilization - overwhelming infections
Felty’s syndrome
Combo of RA (which produces an ulnar deviation of fingers), splenomegaly, and neutropenia
Significant # of Pt’s w/ Felty’s syndrome have monoclonal proliferation of large granular lymphocytes (NK cells)
Causes of neutrophilia
- Acute bacterial infections
- Tissue necrosis (i.e. MI, trauma, burns)
What malignancy is associated w/ eosinophilia?
Hodgkin lymphoma
Lymphocytosis is not seen w/ bacterial infections, except for what?
Bordetella pertussis (whooping cough)
Produces lymphocytosis-promoting factor
Receptor for EBv?
CD21 - on pharyngeal epithelial cells and B lymphocytes
Toxoplasma lymphadenitis
Reactive B lymphocytes result in hyperplasia of the lymphoid follicles and germinal centers (follicular hyperplasia)
Characterized histologically by follicular hyperplasia, proliferation of monocytoid B cells, and epithelioid histiocytes infiltrating lymphoid follicles
What proliferating cells cause hyperplasia of the interfollicular regions and the paracortex
Reactive T lymphocytes - occurs during viral infections
Polycythemia rubra vera is caused by a mutation in?
JAK2
lab findings: increased EPO, decreased O2 saturation, plasma volume unchanged
Secondary polycythemia with appropriate causes of increased EPO
Polycythemia due to inappropriate secretion of EPO
Paraneoplastic syndrome seen with:
Renal cell carcinomas
Hepatocellular carcinomas
Cerebellar hemangioblastomas
Increased reticulocytes in the peripheral smear rules out what type(s) of anemia?
anemias caused by decreased red blood cell production in the bone marrow:
iron deficiency anemia
vitamin B12 deficiency anemia
folate deficiency anemia
aplastic anemia
Hypoplasia of thumbs - associated w/ Fanconi anemia
Peripheral smear obtained from Pt w/ sickle cell disease. Lab shows Hg of 5.6 g/dL. What is the likely cause?
Caused by parvovirus B19
Causes aplastic anemia in children with hemolytic anemia
In otherwise healthy children, parvovirus B19 causes what physical finding?
Slap cheek appearance
15 y/o girl is evaluated due to persistent fever, fatigue, and sore throat. Physical exam reveals splenomegaly and symmetric posterior cervical lymphadenopathy. Peripheral smear results are shown. What is the Dx, what are the cells shown?
Infectious mononucleosis
CD8+ lymphocytes
Where is EPO normally made?
Renal peritubular interstitial cells of the renal cortex
Tumor most associated w/ secondary polycythemia
Renal cell carcinoma