About 10% of people with HCL have this variant form of the disease, representing about 60-75 new cases of HCL-V each year in the U.S. While classic HCL primarily affects men, HCL-V is more evenly divided between males and females. While the disease can appear at any age, the median age at diagnosis is over 70.Similar to B-cell prolymphocytic leukemia (B-PLL) in chronic lymphocytic leukemia, HCL-V is a more aggressive disease. Historically, it has been considered less likely to be treated successfully than is classic HCL, and remissions have tended to be shorter. The introduction of combination therapy with concurrent rituximab and cladribine therapy, though, has shown excellent results in early follow-up. As of 2016, this therapy is considered the first-line treatment of choice for many people with HCL-V.Many older treatment approaches, such as interferon-alpha, the combination chemotherapy regimen "CHOP", and common alkylating agents such as cyclophosphamide, showed very little benefit. Pentostatin and cladribine administered as monotherapy (without concurrent rituximab) provide some benefit to many people with HCL-V, but typically induce shorter remission periods and lower response rates than when they are used in classic HCL. More than half of people respond partially to splenectomy.In terms of B-cell development, the prolymphocytes are less developed than are lymphocytes or plasma cells, but are still more mature than their lymphoblastic precursors.
Umbilical cord prolapse is when the umbilical cord comes out of the uterus with or before the presenting part of the baby. The concern with cord prolapse is that pressure on the cord from the baby will compromise blood flow to the baby. It usually occurs during labor but can occur anytime after the rupture of membranes.The greatest risk factors are an abnormal position of the baby within the uterus and a premature or small baby. Other risk factors include a multiple pregnancy, more than one previous delivery, and too much amniotic fluid. Whether medical rupture of the amniotic sac is a risk is controversial. The diagnosis should be suspected if there is a sudden decrease in the babys heart rate during labor. Seeing or feeling the cord confirms the diagnosis.Management focuses on quick delivery, usually by cesarean section. Filling the bladder or pushing up the baby by hand is recommended until this can take place. Sometimes women will be placed in a knee-chest position or the Trendelenburg position in order to help prevent further cord compression. With appropriate management, the majority of cases have good outcomes.Umbilical cord prolapse occurs in about 1 in 500 pregnancies. The risk of death of the baby is about 10%. However, much of this risk is due to congenital anomalies or prematurity. It is considered an emergency. Signs and symptoms
The first sign of umbilical cord prolapse is usually a sudden and severe decrease in fetal heart rate that does not immediately resolve.