Page 2163 - Hematology_ Basic Principles and Practice ( PDFDrive )

1916 Part XII Hemostasis and Thrombosis

determine the source of the bleeding. 1,3,4,9 Gastrointestinal bleeding when they first began, or did the heavy-flow problems start later in
can be severe with HHT. 25 your life?
Menses up to 7 days in total duration, with 2–3 days of heavy
flow, can be considered normal. 4,5,7,16,29 Although influenced by the
Challenge-Related Bleeding absorbency of the products used, soaking through sanitary products
in less than an hour is suspicious of menorrhagia, as is doubling up
The assessment of challenge-related bleeding is an important part of on products because of heavy flow and gushing and flooding acci-
taking a bleeding history. Bleeding related to accidental trauma may dents. Dysmenorrhea is common among women with bleeding dis-
be more difficult to evaluate than bleeding associated with surgery or orders, and the passage of large blood clots (which reflect increased
dental procedures because accidental trauma often causes bleeding, flow), which is typically painful, suggests the possibility of a bleeding
5
and it is difficult to determine whether the extent of bleeding was disorder. Pictorial bleeding-assessment tools (which are not appli-
7
excessive. In addition, large wounds may continue to bleed until cable to an initial consultation visit) can be helpful to document
sutured. menorrhagia and responses to treatment. 11,29
A history of bleeding with surgical or dental procedures can Postpartum hemorrhage is rarely caused by an underlying bleed-
include being told that there was excessive bleeding by a dentist, ing problem, and it can be complicated by a profound acquired
physician, or other health care worker and/or experiencing excessive coagulopathy, typically with severe fibrinogen depletion. 11–13 On the
oozing or drainage from an incision or extraction sites; wound other hand, excessive or prolonged bleeding after childbirth or
hematomas; delayed wound healing; bleeding requiring repeated pregnancy loss can be problematic for some women with bleeding
surgery, suturing of an extraction site, an admission to hospital, a problems. 9,11 In addition, severe fibrinogen disorders and factor XIII
longer hospital stay, and/or transfer to the intensive care unit; and deficiency compromise carrying a pregnancy to term and need to be
receiving blood transfusions, drugs, and/or factor replacement for excluded if the patient has unexplained pregnancy losses that are
3
hemorrhage control. 1,3,7 Patients may not spontaneously report some associated with hemorrhagic placental abruption (see box on Case
symptoms, such as extensive bruising around surgical incisions. 4: Evaluation of an Isolated Symptom—Recurrent Pregnancy Loss
Occasionally an operative report or other medical document provides With Bleeding).
important confirmation that there was abnormal bleeding with During the first week after childbirth, the bleeding (lochia) is
surgery (e.g., an operative note that documents generalized oozing typically characterized by brighter red flow than a normal period.
1,3
during a procedure and greater-than-expected total blood loss). Afterward, the flow usually lightens and continues for up to 6 weeks
Iatrogenic reasons (e.g., oozing vessels that were not cauterized or postpartum. Flow can be heavier, or persist longer, in women with
ligated) should be considered when there is a history of an isolated bleeding disorders. 9,11
bleeding episode.
Many individuals undergo dental extractions at some point in
their life. Bleeding that persists beyond the first day, or that becomes Anemia Related to Bleeding
problematic one or more days after a dental extraction, should be
1,3
considered suggestive of a bleeding disorder. Severe bleeding with A history of anemia and/or prior treatment with iron replacement is
dental cleaning should be considered suggestive of a congenital or an frequently reported by women with bleeding disorders. 3,16 Pallor of
acquired bleeding disorder (e.g., von Willebrand disease or a platelet the palms is often observed when the hemoglobin is below 10 g/dL.
function disorder). Anemia is uncommon in individuals with bleeding disorders unless
In individuals with a moderate-to-severe bleeding problem, bleed- there is acute bleeding or chronic persistent bleeding leading to iron
ing after surgery, dental procedures, or a severe throat infection can deficiency that compromises red cell production. Many women with
lead to airway compromise, whereas bleeding from a surgical or bleeding disorders and menorrhagia have low iron stores, but not
traumatic limb injury can lead to compartment syndrome. 1,3,28 anemia. Low iron stores may also reflect ongoing gastrointestinal
bleeding (overt or occult), which if present, requires investigation
even if there is a known congenital or acquired bleeding problem.
Bleeding Symptoms Restricted to Women Some bleeding disorders (e.g., platelet disorders from GATA1 muta-
tions) are associated with anemia and thrombocytopenia.
Women with bleeding disorders experience more bleeding than men
because of the hemostatic challenges associated with menses and
childbirth. 16,29 Such women are also at increased risk for developing Joint Bleeds and Muscle Bleeds
endometriosis and hemorrhage from ovarian cysts. 16,29 They may also
report troublesome bruising or bleeding with sexual activity. Joint bleeds and bleeding into muscles (e.g., iliopsoas bleeds), which
Menorrhagia is a fairly common manifestation of bleeding disor- are uncommon bleeding symptoms, suggest a severe coagulation
ders, and the hemostatic cause can be von Willebrand disease, a
platelet disorder, or a defect in coagulation or fibrinolysis. 7,15,16,29
However, menorrhagia can arise from other causes, such as Case 4: Evaluation of an Isolated Symptom—Recurrent Pregnancy Loss
fibroids. 3,17,29 Menorrhagia from inherited bleeding disorders is often With Bleeding
long standing, but it can be influenced by treatments. Accordingly,
it is important to ask about menses when on, and not on, treatment. A 32-year-old woman was referred for evaluation of a low fibrinogen
Menorrhagia can develop as a manifestation of an acquired bleeding level in the setting of acute placental abruption, resulting in a third
pregnancy loss (this time in the third trimester). She had no prior
problem (e.g., from acquired von Willebrand disease or anticoagulant bleeding history apart from having suffered three placental abruptions
therapy for deep vein thrombosis). associated with severe bleeding that required transfusion. The family
In general, it is more helpful to ask women quantitative or history was negative for bleeding problems. She had previously been
categorical questions about menses, rather than qualitative questions investigated for thrombophilia but had not been tested for a bleeding
(e.g., are/were your menstrual periods heavy?). Questions to consider disorder. The low fibrinogen level persisted over many months (levels
include the following: How many days of bleeding do you have with of approximately 90 mg/dL), suggesting that the defect was inherited.
your typical menstrual periods? How many days of this bleeding She received fibrinogen concentrate for two subsequent pregnancies,
were heavy flow? On your heavy days of flow, did you soak through which she carried to term and delivered without bleeding problems.
sanitary products in an hour or less? What treatments have you This case illustrates the need to consider inherited disorders when the
bleeding symptoms are unusual and severe, even if there is only one
taken for heavy periods? When you were not on treatment, how bleeding symptom. It also illustrates that prognosis is dependent on
many days of bleeding (and how many days of heavy flow) did you diagnosis and treatment.
have with a typical menstrual period? Were your periods like this