Term
| Hematopoiesis begins in the |
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Definition
•Yolk sac during first month of development
•Liver & spleen at third month of development
•Bone marrow around the fourth month
•Liver & spleen hematopoeisis ceases at birth |
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Term
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Definition
Group AB recipient is universal, since they do not make anti-AB antibody
•If patient is 0 type, only 0 type blood can be safely given
•If patient is AB, A or B can be used, as well as 0 packed cells |
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Term
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Definition
•Direct test looks for IgG, which crosses placenta, rarely agglutinates cells, is optimal at 37 °C, is not naturally occurring
•Test for any autoimmune hemolysis
•Patient’s RBCs tested with Coombs' reagent = antihuman IgG
•If the patient is hemolyzing, the Coombs' reagent binds to the patient's IgG which is attached to the patient’s own RBCs, causing agglutination |
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Term
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Definition
•Indirect looks for IgM, which cannot cross placenta, agglutinates cells, works at 4-20 °C & naturally occurs
•Patient's serum (recipient) is incubated with RBCs from the donor of known antigenicity
•
Used to diagnose atypical, unexpected antibodies in the patient's serum & for pre-transfusion compatibility |
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Term
| Complications of Blood Transfusion |
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Definition
| Associated with fever, chills, facial flushing, chest/back pain, heat/pain at infusion site, dyspnea, hypotension, agitation, hemoglobinuria |
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Term
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Definition
•Produces pancytopenia, petechiae/hemorrhage, pallor, weakness, infection
Etiology is usually idiopathic, however drugs (chloramphenicol), toxins (benzene, etc.), infections (parvovirus B19 is most common, also hepatitis B or C), radiation, paroxysmal nocturnal hemoglobinuria |
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Term
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Definition
Aplastic Anemia
Light micrograph of a bone marrow smear showing fewer red blood cells (small grey) than would be expected in normal marrow. The larger cells (blue) are white blood cells |
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Term
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Definition
Bone Marrow Smear
(Megaloblastic Anemia) |
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Term
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Definition
| Hypercellularbone marrow in megaloblasticanemia |
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Term
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Definition
•Impaired DNA synthesis with normal RNA & protein synthesis
•Causes include B12 deficiency, folate deficiency, chemotherapy
•Pathognomonic blood smear is the hypersegmentedneutrophils (macropolycytes) |
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Term
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Definition
clusters of DNA in circulating erythrocytes seen in severe cases of megaloblasticanemia
Megaloblastic Anemia |
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Term
| B12 (cyanocobalamin) deficiency |
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Definition
•Associated with megaloblastic anemia and neuropathies secondary to defective myelin synthesis
•Often due to lack of intrinsic factor (needed by mucosal cells in terminal ileum for B12 absorption) |
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Term
| Megaloblastic Anemia Diagnosis |
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Definition
•Serum methylmalonic acid & homocysteine levels are more sensitive than pure folate & B12 levels
•Both methylmalonic & homocysteine increase in B12 deficiency |
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Term
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Definition
•Iron is transported via transferrin to marrow, used by developing RBCs to make hemoglobin & is stored in macrophages
•Reduction of iron, increase in TIBC, reduction of ferritin, dramatic reduction of iron shown on marrow stains |
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Term
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Definition
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Term
| Anemia of Chronic Disease |
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Definition
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Despite the reduction in the level of iron, no concomitant increase in TIBC (unknown reason) |
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Term
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Definition
•Produces a hypochromic microcytic anemia due to lead inhibition of heme synthesis
•Classic diagnostic findings include: Bruton's lines = blue/gray discoloration at gumlines; Basophilic stippling of red cells (blue dots in red cells) |
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Term
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Definition
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Term
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Definition
•Produces a hypochromic microcytic anemia due to lead inhibition of heme synthesis
•Classic diagnostic findings include: Bruton's lines = blue/gray discoloration at gumlines; Basophilic stippling of red cells (blue dots in red cells) |
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Term
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Definition
•Usually Mediterranean or black ethnicity
•Manifestations include hepatosplenomegaly, anemia, frontal bossing due to extramedullary hematopoiesis, hypercellular marrow, iron overload (secondary to transfusions), recurrent infection and possible early death |
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Term
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Definition
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Term
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Definition
•Sickle cells clog microcapillaries -> vaso-occlusive findings, pain crisis, myocardiopathy, infarcts of bone/CNS/lungs/kidneys, priapism & autosplenectomy due to splenic infarct
•Autosplenectomy increased susceptiblity to encapsulated bacteria
•Intravascular hemolytic episodes can cause gallstones in children, teens & young adults |
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Term
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Definition
•Substitution of lysine for glutamine
•Homozygous CC causes mild chronic anemia, less severe than sickle S |
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Term
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Definition
•Decreased platelets in the blood
•Presents with petechiae, epistaxis, CNS bleeds & GI bleeds
•Bleeding time elevated at counts <50,000
•Clinically significant bleeds start at counts <20,000
•CNS bleeds occur with counts <10,000 |
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Term
| Idiopathic thrombocytopenic purpura (ITP) |
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Definition
•An autoimmune disorder of autoantibody-mediated platelet destruction
•Manifestations include: petechiae, purpura, epistaxis, with normal white & red cell morphology on peripheral blood smear
•In children follows URI (upper respiratory infection) and is self-limiting, in adults it is chronic |
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Term
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Definition
•An X-linked deficiency in factor VIII, the most common hemophilia type
•Labs: Increased PTT, normal PT, normal bleeding time, decreases factor VIII levels |
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Term
| Hemophilia B (Christmas disease) |
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Definition
•X-linked factor IX deficiency, also with variable disease severity
•Lab shows increased PTT, normal PT, normal bleeding time, low factor IX levels, normal VIII levels |
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Term
| von-Willebrand factor (vWF) deficiency |
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Definition
•Most common inherited bleeding disorder (more common than hemophilia)
•vWF deficiency presents with episodic increased bleeding time & ecchymoses, with normal PT (thrombin time)/PTT (partial thromboplastintime)
•Most commonly autosomal dominant and there are three different types of this condition |
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Term
| von-Willebrand factor Type I |
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Definition
| due to secretion of functional vWF |
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Term
| von-Willebrand factor Type II |
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Definition
| due to secretion of dysfunctional vWF |
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Term
| von-Willebrand factor Type III |
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Definition
| autosomal recessive with dysfunctional vWF |
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Term
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Definition
•Acts as a vitamin K analogue to inhibit carboxylation of factors II, VII, IX, X, as well as proteins C & S
•Because Coumadin also interferes with protein C synthesis, & protein C has a very short half-life, initial administration of Coumadin can cause a fulminant (sudden) skin necrosis secondary to capillary thrombosis |
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Term
| Acute Myelogenous Leukemia (AML) |
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Definition
Risk factors
•Myelotoxic agents: Benzene, and chemotherapeutic alkylating agents are most important factors
•Radiation
•Trisomy 21 (Down Syndrome) has propensity to lead to AML, though most cases with this syndrome are acute lymphoblastic leukemia |
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Term
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Definition
| (structures characteristic of myeloblasts) are found in AML |
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Term
| Chronic Myelogenous Leukemia(CML) |
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Definition
•Philadelphia chromosome is found in all cases of CML and it is pathognomic
•Shortened chromosome 22 and translocation of abl from 9 to bcr on 22 (most common and classic translocation)
•abl is fused to bcr on Chromosome 22, such that bcr drives the over expression of the abl gene
•A neoplastic disorder of multipotential hemopoeitic stem cells that principally involves granulocytic cell lineage
•Constitutes 20% of all leukemia
•Patients display pronounced granulocytosis and immaturity of granulocytic elements, anemia, thrombocytosis, basophilia and splenomegaly
•Most common in middle-age and elderly adults |
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Term
| Chronic Myelogenous Leukemia(CML) Diagnosis |
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Definition
•Normally peripheral blood is devoid of myelocytes
•CML shows fewer than 30% myeloblasts, otherwise the diagnosis is changed to AML |
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Term
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Definition
•Occurs in a bimodal age distribution, young men (women for nodular sclerosis type, see below) & geriatric population
•EBV infection is present in up to 50% of cases
•Signs and symptoms resemble inflammatory disorder, classic Pel-Epstein fever(fevers wax & wane over weeks), chills, night sweats, weight loss, leukocytosis, in some patients symptoms worsen with alcohol intake |
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Term
| Reed-Sternberg (RS) cells |
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Definition
•Possibly the malignant cell of Hodgkin's
•Classically appear as binucleated giant cells ("owl eyes")with eosinophilic inclusions
•One variation is Lacunar cell, a mononucleated giant cell
•Disease severity is proportional to number of R-S cells seen in tumor cells |
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Term
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Definition
| Reed-Sternberg (RS) cells(Oweleye) |
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Term
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Definition
•
Patients present with headache, vertigo, diplopia, retinal hemorrhages, strokes, angina, claudication (pain upon physical activity), early satiety, splenomegaly, gout, pruritus after showering, plethora |
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Term
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Definition
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Term
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Definition
Portal hypertension secondary to splenic vein thrombosis
(Polycythemia Vera) |
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Term
| Non-Hodgkin’s Lymphoma Follicular (nodular) type |
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Definition
•Rare in children, better prognosis than diffuse counterpart
•B-cell type
•Patients with small cells do better than large cells |
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Term
| Non-Hodgkin’s Lymphoma Diffuse type |
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Definition
•More aggressive than nodular
•Either B-cell or T-cell type
•Most aggressive NHLs; all are histologicallydiffuse types |
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Term
| Non-Hodgkin’s Lymphoma (NHL) Low grade |
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Definition
small lymphocytic, follicular small cleaved cell, follicular mixed small cleaved
Folicular |
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Term
| Non-Hodgkin’s Lymphoma (NHL) Intermediate grade |
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Definition
follicular large cell, diffuse small cleaved cell, diffuse mixed/small/large cell types
Mixed |
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Term
| Non-Hodgkin’s Lymphoma (NHL) High grade |
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Definition
highly aggressive and are always diffuse and include immunoblastictype seen in immunocompromised and Lymphoblastic involves mediastinum & bone marrow, is terminal deoxynucleotidyltransferase(TdT) positive & has T-cell markers
Diffuse |
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Term
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Definition
Small Noncleaved cell of NHL
Classic histologic description is the "starry sky pattern," caused by dark background of densely packed lymphocytes (sky) with light colored spots in them caused by scattered macrophages (the stars)
•Translocation of c-mycfrom chromosome 8 to chromosome 14 Igheavy chain locus |
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