Case discussion:
A 25-year-old Caucasian female with no significant past medical history
except for well controlled bronchial asthma presented to us with two-day
history of worsening petechiae that initially started on her face and
lower extremities and eventually spread to back, chest, abdomen and
upper extremities. She also complained of intermittent epistaxis.
Vital signs on initial presentation including blood pressure, heart rate
and oxygen saturation were normal. Physical examination revealed
extensive bruising over her back, chest and extremities along with
scattered petechiae all over the body. Laboratory findings on
presentation include WBC of 11x109/L, hemoglobin (Hb)
of 11g/dl and severe thrombocytopenia with a platelet count of
1x109/L. Prothrombin time (PT/INR), activated partial
thromboplastin time (aPTT), fibrinogen, Lactate dehydrogenase (LDH),
absolute reticulocyte count and haptoglobin levels resulted normal.
Peripheral blood smear showed profound thrombocytopenia with no evidence
of schistocytes. RT-PCR testing for SARS CoV-2 infection was negative.
Autoimmune and infectious workup including antinuclear antibody (ANA)
screening, coombs testing, HIV ELISA and serology for hepatitis B, and C
were negative. Ultrasound of the abdomen did not show evidence of
splenomegaly or liver parenchymal changes.
Her most recent hematological labs including Hb, platelet count from
three months ago during routine primary care visit were within normal
limits. She neither reported personal or family history of bleeding
disorders nor required blood transfusions prior to this presentation. 26
days prior to this presentation she had received her second dose of
Moderna mRNA vaccination.
Given her clinical presentation, negative infectious and autoimmune
workup, normal hemolysis and coagulation studies along with absence of
other identifiable underlying etiology, she was presumed to be having
immune mediated thrombocytopenic purpura (ITP) and was initiated on
dexamethasone 40mg daily for four days along with intravenous
immunoglobulin (IVIG) 1g/kg for two days. She continued to have
clinical worsening during the second and third day of hospitalization
and developed multiple bleeding sites including gross hematuria,
hematochezia, subconjunctival hemorrhage. Her hemoglobin dropped from
11g/dl on presentation to 6.4g/dl. She was initiated on tranexamic acid
for bleeding control and supportive care with platelet transfusions. Her
platelet counts continued to remain low (5x109/L)
despite receiving 4 days of dexamethasone, 2 days of IVIG and platelet
transfusion support. She continued to have intermittent epistaxis
however hematuria and rectal bleeding started improving with Tranexamic
acid initiation.
As there was no improvement in platelet counts with IVIG and
dexamethasone, on day 7 she was initiated on mycophenolate mofetil (MMF)
1 gram twice daily and Romiplostim 1 mcg/kg weekly. On day 12 her
platelet counts for the first time improved to
13x109/L. Hb remained stable around 11g/dl. She was
discharged after receiving second dose of Romiplostim 3mcg/kg on day 14.
During her first outpatient visit on day 21 her platelet counts improved
to 140x109/L. She went on to receive third dose of
Romiplostim at 3mcg/kg. She continued to have no active sites of
bleeding. During fourth week follow up, platelet count recovered to
268x109/L and MMF was tapered down to 500mg twice
daily and Romiplostim to 2mcg/kg. By fifth week, her platelet counts
continued to improve to 324x109/L and MMF was tapered
to 250mg twice daily and Romiplostim to 1mcg/kg. She continued to have
normal and stable platelet counts and eventually MMF and Romiplostim
were discontinued.