52 Fragmentation Hemolytic Anemia

Question 1

Two-thirds of HELLP patients are diagnosed antepartum, usually between_______ weeks

Answer 1

27 and 37 weeks

Question 2

Risk factors for HELLP syndrome

Answer 2

European ancestry, multiparity, maternal age (older than age 34 years), and a personal or familial history of the disorder

Question 3

In a preeclamptic pregnancy, the second wave fails to penetrate adequately the spiral arteries of the uterus, perhaps as a result of reduced placental expression of ______

Answer 3

Syncytin

Question 4

Functions as an antiangiogenic protein because it binds to vascular endothelial growth factor (VEGF) and placental growth factor (PGF) and prevents their interaction with endothelial cell receptors.

Answer 4

Soluble vascular endothelial growth factor receptor-1 (sVEGF receptor-1, or sVEGFR-1)

**Ratio of sVEGFR-1 to PGF levels is increased. Result is maternal glomerular endothelial cell and placental dysfunction

Question 5

Ninety percent of patients with HELLP syndrome present with

Answer 5

Malaise and right upper quadrant or epigastric pain

Fever is not typically seen.

Question 6

The ratio of ________(an isoenzyme found specifically in the liver) to total LDH is elevated in proportion to the severity of HELLP.

Answer 6

LDH-5

Question 7

The high LDH seen in HELLP is most likely the result, principally, of ______________ rather than hemolysis

Answer 7

Liver damage

Question 8

Liver enzymes usually return to normal within _______days postpartum.

Answer 8

3–5 days postpartum

Question 9

Mississippi triple-class system: based on platelet count

Class 1:
Class 2:
Class 3:

Answer 9

Class 1: less than 50 × 109/L ***highest incidence of perinatal morbidity and mortality

Class 2: between 50 and 100 × 109/L

Class 3:greater than 100 × 109/L

Question 10

Seen in the last trimester or postpartum and presents with thrombocytopenia and right upper quadrant pain, but the levels of AST and ALT only rise to one to five times normal, and the PT and activated partial thromboplastin time (aPTT) are both prolonged

Answer 10

Acute fatty liver of pregnancy

Question 11

Indications for delivery in HELLP

Answer 11

Severe disease presentation, maternal DIC, fetal distress, and a gestational age greater than 32 weeks with evidence of lung maturity

Question 12

TRUE OR FALSE

The use of dexamethasone has fallen out of favor after large randomized trials found that it did not reduce the duration of hospitalization, amount of blood products transfused, maternal complications, or time to normalization of laboratory abnormalities

Answer 12

TRUE

The use of dexamethasone has fallen out of favor after large randomized trials found that it did not reduce the duration of hospitalization, amount of blood products transfused, maternal complications, or time to normalization of laboratory abnormalities

Question 13

TRUE OR FALSE

Plasma exchange cannot arrest or reverse HELLP syndrome when used antepartum but may minimize hemorrhage and morbidity when used peripartum.

Answer 13

TRUE

Plasma exchange cannot arrest or reverse HELLP syndrome when used antepartum but may minimize hemorrhage and morbidity when used peripartum.

Question 14

Indications of liver transplantation in HELLP

Answer 14

Large hematomas or total hepatic necrosis

Question 15

Hepatic hematoma is usually present in the ____________________

Complain of right-sided shoulder pain and are found to be in shock with ascites or pleural effusions

Answer 15

Anterior superior portion of the right lobe of the liver

Question 16

TRUE OR FALSE

One-third of all babies born to mothers with HELLP have thrombocytopenia

Answer 16

TRUE

One-third of all babies born to mothers with HELLP have thrombocytopenia

Fetal morbidity and mortality rates are each approximately 15%.

Question 17

DISSEMINATED MALIGNANCY

80% of the tumors are mucinous adenocarcinomas of either the ____________________

MAHA is more likely to be associated with metastatic malignant disease than with localized cancers or benign tumors.

Answer 17

Stomach (55%)
Breast (13%)
Lung (10%).

Question 18

Two distinct mechanisms in Cancer-associated MAHA:

Answer 18

(a) DIC with intravascular occlusions (often partial) of small vessels by platelet-fibrin thrombi or

(b) intravascular tumor emboli.

Question 19

TRUE OR FALSE Cancer-associated MAHA:

Although the reticulocyte count can be high, it is an unreliable measure of hemolysis because extensive replacement of the marrow by metastatic tumor may prevent the reticulocytosis expected with MAHA.

Answer 19

TRUE’

Although the reticulocyte count can be high, it is an unreliable measure of hemolysis because extensive replacement of the marrow by metastatic tumor may prevent the reticulocytosis expected with MAHA.

Question 20

Autoimmune hemolytic anemia secondary to malignancy is seen in:

Answer 20

Lymphoproliferative disease
Occasionally seen with carcinoma of the stomach, colon, breast, and cervix

Question 21

Anemia was subsequently shown to be caused by erythrocyte shearing and fragmentation as the red cells traversed the turbulent flow through or around the prosthetic valve.

Answer 21

HEART VALVE HEMOLYSIS

Question 22

Factors can increase the chance of valvular hemolysis

Answer 22

Presence of central or paravalvular regurgitation, placement of small valve prostheses with resultant high transvalvular pressure gradients, and regurgitation because of bioprosthetic valve failure, seen especially when the valve is more than 10–15 years old

Question 23

A transvalvular pressure gradient of more than 50 torr can generate shearing forces exceeding 4000 dynes/cm2, more than the ___________ dynes/cm2 usually needed to cause red cell fragmentation.

Answer 23

3000 dynes/cm2

Question 24

The presenting complaint is passage of dark urine immediately after physical exertion in the upright position, occasionally accompanied by nausea, abdominal cramps, aching in the back or legs, a “stitch in the side,” or a burning feeling in the soles of the feet

Answer 24

MARCH HEMOGLOBINURIA

Question 25

A syndrome that usually develops in early childhood and is characterized by thrombocytopenia, MAHA, consumptive coagulopathy, and hypofibrinogenemia caused by an enlarging kaposiform hemangioendothelioma or tufted angioma.

Answer 25

Kasabach-Merritt phenomenon