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Pathology - Systemic Exam 1b Heme
Pathology - Systemic Exam 1b Heme
42
Pathology
Professional
05/10/2013

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Term
3 Beta Thalassemia Clinical Syndromes ?
Definition
1. B Thal Major

2. B Thal Minor / Trait

3. B Thal Intermedia
Term
Characteristics of Beta Thalassemia Major Syndrome ?
Definition
Usually immigrants from Mediterranea, Africa, southeast Asia

Presentes 6 to 9 months after birth

HbF remains elevated

HbA2 may be normal, low, or high

Prominent facial bones
Hepatosplenomegaly and Splenomegaly
Hemosiderosis and Hemochromatosis
Term
Thallasemias are what type of anemia ?
Definition
Hypochromic microcytic anemia
Term
Characteristics of Beta Thalassemia Minor ?
Definition
Mild hypochromic microcytic anemia

Usually asymptomatic

Increase HbA2 with normal or increase HbF

Necessary to recognize Beta Thalassemia trait to avoid treating as iron deficiency anemia

Genetic counseling
Term
Trt for Beta Thalassemias ?
Definition
Regular blood transfusions

Iron chelators to prevent iron overload and cardiac failure

Bone marrow Transp. may be curative for Beta Thalassemia Major
Term
The four alpha globin genes are located on chromosome ?
Definition
16
Term
4 variants of Alpha Thalassemia ?
Definition
Silent carrier - one alpha gene deleted

Alpha Thal Trait

HbH (Hemoglobin H disease)

(Hb Barts) Hydrops Fetalis - all 4 alpha genes deleted, incompatible with life
Term
What is PNH ?
Definition
Rare, acquired, stem cell disorder associated with periodic hemolysis, due to mutations of X-linked gene PIGA (phosphatidylinositol glycan class A.

PIGA inhibits complement (absence of CD55, CD59, C8 binding protein) causes hemolysis
Term
Leading cause of death in PNH ?
Definition
Thrombosis
Term
Trt for PNH ?
Definition
Immunosuppression
Bone marrow transplant
Term
What type of anemia can you get from mechanical damage to RBCs, like with valve prostheses ?
Definition
Microangiopathic hemolytic anemia
Term
What anemias can you get from

A) Defective DNA synthesis ?

B) Defective heme synthesis ?

C)Defective globin synthesis ?
Definition
A) Megaloblastic anemia, b/c Vit B12 and folate are needed to make thymidine, one of the bases of DNA

B) Iron deficiency anemia

C) Thalassemias
Term
Vitamin B12 gets absorbed where in body ?
Definition
Ileum
Term
Decrease in intrinsic factor and then Vit B12 deficiency causes what anemia ?
Definition
Pernicious anemia (type of megaloblastic anemia)

Low serum B12
Normal folate
Increase homocysteine and methylmalonic acid
Term
Schilling test for ?
Definition
Diagnose pts. with pernicious anemia if they cannot absorb oral dose of Vit B12
Term
Any neurological deficits in folate deficiency ?
Definition
No
Term
Most common cause of anemia worldwide ?
Definition
Iron deficiency anemia
Term
Hepcidin ?
Definition
Peptide made in liver when liver iron stores are too high. It inhibits iron absorption in duodenum.
Term
Free iron is toxic ?
Definition
Yes, so most iron in body is bound as ferritin
Term
"Poikilocytosis" with "pencil cells" seen in what anemia ?
Definition
Severe cases of iron deficiency anemia
Term
Increase RDW and Increase TIBC, and decrease in other measures seen in what anemia ?
Definition
Iron Deficiency anemia
Term
Most common anemia in hospitalized pts. ?
Definition
Anemia of chronic disease
Term
Congenital form of aplastic anemia is called ?
Definition
Fanconi anemia
Term
Aplastic anemia is actually a ?
Definition
** Pancytopenia **
Term
Bone marrow biopsy for aplastic anemia shows what ?
Definition
Very hypocellular, mostly only fat cells seen
Term
In what anemia do you see dacrocytes (tear drop cells) ?
Definition
Myelophthisic anemia

Also in myelofibrosis
Term
What is polycythemia / erythrocytosis ?
Definition
Abnormally elevated RBC concentration and Hb levels

Can be relative or absolute

Absolute:
Primary - Ex is Polycythemia vera

Secondary -
a) Appropriately high EPO levels:
b) Inappropriately high EPO levels: tumors or cancer
Term
Prolonged bleeding time indicates ?
Definition
Platelet defect (reduced number / function)
Term
Thrombin time tests ?

Diff. from prothrombin time (PT)
Definition
Fibrinogen
Term
Thrombocytopenia is ?
Definition
Decreased numbers of platelets

Causes prolonged bleeding time

A lot of causes
Term
Trf for immune thrombocytopenia (ITP) ?
Definition
Immunosuppresion (steroids)
Splenectomy
Term
Most common inherited bleeding disorder ?
Definition
Von Willebrand Disease

Mostly Autosomal Dominant
3 types:
Type 1 - Most cases. Mucosal bleeding like dental cavity bleeding

Type 2 - Mild bleeding

Type 3 - A lot of bleeding
Term
Hemophilia A is ?
Definition
Factor 8 deficiency

Most common hereditary disease with serious bleeding

X-linked recessive

Prolonged PTT

Trt: Recombinant Factor 8 infusions
But small percentage develop Factor 8 antibodies, and there is risk of trasmission of viral disease
Term
Agranulocytosis =
Definition
Severe neutropenia

Most commonly drug induced

Empirical trt with broad spectrum antibiotics

In predictable neutropenia, such as following chemotherapy, drug regimes usually includes G-CSF
Term
Leukocytosis is
Definition
Increased leukocytes or WBCs in blood

Can be increased neutrophils, basophils, eosinophils, lymphocytes, or monocytes
Term
Basophilic leukocytosis almost always indicates ?
Definition
Myeloproliferative neoplasm (usually CML, chronic myeloid leukemia)
Term
Dohle bodies are seen in
Definition
Peripheral blood during sepsis and acute infections ?

Are small blue smudges inside cells
Term
90 % of Non-Hodgkin Lymphomas are
Definition
B-cell lymphomas
Term
Most common site of Marginal Zone Lymphoma (Maltoma) ?
Definition
Stomach, superimposed on H. Pylori gastritis
Term
Which neoplasm gives you these symptoms :

Painless rubbery enlargment of one or more lymph nodes
Pain in involved nodes after drinking alcohol
Cutaneous anergy (itching)
Definition
Hodgkin's Lymphoma
Term
4 stages of Ann Arbor Clinical Staging is for ?
Definition
Hodgkin's Lymphoma
Term
Lacunar cells (type of Reed-Sternberg cells) are present in what lymphoma ?
Definition
Nodular Sclerosis subtype of Hodgkin's Lymphoma
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