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ANGELES UNIVERSITY FOUNDATION

Angeles City
COLLEGE OF ALLIED MEDICAL PROFESSIONS

Hematology LABORATORY

Case analysis

Paroxysmal
Cold
hemoglobinuri
a
Submitted by:
Group 9
BS MT 3-C
Group Leader: David, Kyla Denise
Members: De Jesus, Kim Paula
Leongson, Shirlene Anne
Malaban, Kelvin

Submitted to:
Maam Genevieve Dizon
Sir Jeshua Caleb Miole
HEMA2A Laboratory Professors

January 19, 2017


CASE SUMMARY
81-year old woman with a medical history of hypertension and dementia presented
to the emergency department in January (winter time) with complaints of
progressively worsening abdominal pain and vomiting for three days. Recently,
admitted to another hospital with similar complaints and pink-coloured urination.
During this admission, she was found to have abdominal tenderness, dry mucus
membrane, pallor and icterus. Laboratory studies revealed the following:
Hematocrit: 18%
MCH: 37 pg
WBC count: 5.1/cumm
PBS: RBC fragment and polychromasia
Reticulocyte: 12.5%
DAT: Positive

GUIDE QUESTIONS
1. What possible condition does the patient present with?
The possible condition of the patient is Paroxysmal Cold Hemoglobinuria (PCH). It is
an acute form of cold-reactive hemolytic anemia and may be idiopathic or secondary.
Paroxysmal cold hemoglobinuria is the least common type of AIHA. It is transient
and self-limiting but can produce serious hemolysis of erythrocytes. It occurs almost
exclusively in children in association with viral disorders. Erythrocyte destruction is
the result of a cold-reacting, IgG autoantibody termed autohemolysin.

2. Explain the pathophysiology of the disorder.


This autoagglutinin, the Donath-Landsteiner antibody with anti-P specificity, only
attaches to erythrocytes at cooler temperatures and then activates complement in
warmer temperatures. This type of antibody activity is called biphasic hemolysis.
The anti-P autoantibody, also called the Donath-Landsteiner antibody, is a
complement-binding IgG hemolysin with specificity for the P antigen on RBCs. The
anti-P autoantibody is biphasic in that at cold temperatures it binds to the P antigen
on RBCs and partially activates complement (C1 to C4), but full complement
activation (C3 through C9) and hemolysis occur only upon warming to 37 C.
Exposure to cold temperatures is not required for the hemolytic manifestations in
vivo, however, and the reasons for this have yet to be explained. The anti-P
autoantibody binds antigen optimally at 4 C and has a thermal amplitude of less
than 20 C. At warmer temperatures, the anti-P autoantibody dissociates from the
RBCs. The titer is usually less than 1 : 64.
3. What are the clinical signs and symptoms for this condition?

acute fever
malaise, and back, leg, and/or abdominal pain 1 to 2 weeks after an upper

respiratory tract infection


pallor
jaundice
dark urine due to hemoglobinuria
rapidly progressing and severe anemia,

4. Give other laboratory findings that correlate to this disorder?

hemoglobin levels dropping below 5 gm/dL


reticulocytosis is typical but can be preceded by reticulocytopenia
The peripheral blood film shows: polychromasia,and spherocytes,
PBS may also display: schistocytes, nucleated RBCs, anisocytosis,

poikilocytosis, and erythrophagocytosis


DAT positive

5. What treatments may be given to patients with this disorder?

PCH is severe but self-limited and resolves in several days to a few weeks
with an excellent prognosis. In most patients, the anemia is severe and can
be life-threatening, so transfusion is usually needed until the symptoms

resolve.
The mainstay of treatment for paroxysmal cold hemoglobinuria is supportive

care and the avoidance of cold exposure.


Once hemolysis is suspected, folic acid 1 mg/d orally should be instituted to
help with erythropoiesis. Folic acid is lost via the hemolytic process and hence

needs to be replenished.
Administer warmed, packed RBC transfusions for life-threatening hemolysis
and symptomatic anemia. Utilizing washed RBC units has not been proven to
improve transfusion safety, but this can be performed if patient's condition

remains refractory to standard warmed products.


Treat underlying secondary conditions with appropriate medical therapy.

PERTINENT INFORMATION
Donath-Landsteiner test
-

This is a test used to confirm the diagnosis of PCH. This involves the
collection of a fresh blood sample from a patient which is maintained at 37C.
The serum is separated into three sets of three test tubes A1-A2-A3, B1-B2B3, and C1-C2-C3. These are incubated at various temperatures with group
O RBCs that express the P antigen. In this test, 1 and 2 of each set would
contain 10 drops of patients serum and 3 would 2 and 3 would contain 10
drops of fresh normal serum as a complement source. 50% suspension of
washed P+ RBCs is added to each tube and mixed. After mixing, three tubes
are placed in an ice bath for 30 minutes and then incubated at 37C for one
hour. The three B tubes are placed in a melted ice bath for 90 minutes. The
three C tubes are kept at 37C for ninety minutes as well. After the incubating
the tubes, check for hemolysis in the supernatant.

Results:
Incubation

Tube 1

Tubes 2

Tubes 3

Phases
Ice bath followed

by 37C
Ice bath only

37C only

REFERENCES
Turgeon, M., Clinical Hematology: Theory and Procedures (Fifth Edition) p. 203
Rodak, et al., Hematology: Clinical Principles and Applications (Fourth Edition) p.
359
Harmening, Denise, Modern Blood Banking & Transfusion Practices (Sixth Edition)
p. 449-451
Steininger, et al., Clinical Hematology: Principles, Procedures, Correlations p. 274275
Turgeon, M., Immunology & Serology in Laboratory Medicine (Fifth Edition) p. 395
http://emedicine.medscape.com/article/200947-treatment (accessed 01/14/17)