Surreal illustration of APL in pregnancy

Unexplained Postpartum Hemorrhage? Don't Overlook This Rare Leukemia

"Acute promyelocytic leukemia (APL) can present unexpectedly, underscoring the importance of a thorough evaluation even in seemingly routine postpartum complications."


Acute promyelocytic leukemia (APL) is a unique form of acute myeloid leukemia (AML) distinguished by a specific genetic abnormality: the t(15;17) chromosomal translocation. While rare in the general population, its presentation during pregnancy poses unique diagnostic and therapeutic challenges. It’s estimated that only 50 cases have been reported in medical literature.

Postpartum hemorrhage (PPH) is a well-recognized complication following childbirth, but what happens when the bleeding is not due to the usual suspects? A recent case highlights an unusual presentation of APL: secondary PPH. This underscores the importance of considering rare hematological disorders when common postpartum problems arise.

This article delves into a case study where APL mimicked typical postpartum complications, emphasizing the need for vigilance and comprehensive diagnostic approaches, especially when initial treatments fail to resolve the issue.

The Case: When Postpartum Bleeding Masked a Serious Condition

Surreal illustration of APL in pregnancy

A 30-year-old woman, who had a normal vaginal delivery, was readmitted six days postpartum with complaints of excessive vaginal bleeding and bleeding gums. Initial ultrasound findings suggested retained products of conception, a common cause of secondary PPH. However, her condition didn't improve with standard treatments.

Initial blood tests revealed a hemoglobin level of 8.8 g/dL, a total leukocyte count of 6.5 × 109/L, and a significantly reduced platelet count of 44 × 109/L. The differential leukocyte count showed 2% blasts and 65% promyelocytes, raising suspicion for a hematological malignancy.

  • Key Indicators: Looked pale with no fever.
  • Unusual Findings: No Icterus, pedal edema or lymphadenopathy
  • Hematological Clues: Hemoglobin 8.8 g/dL, Leukocyte count 6.5×10°/L, Platelet count 44×109/L
A peripheral blood smear confirmed the presence of abnormal promyelocytes, some containing characteristic “faggot cells” (bundles of Auer rods). These promyelocytes stained strongly positive for myeloperoxidase, leading to a presumptive diagnosis of AML, specifically suggesting APL. Unfortunately, the patient's condition deteriorated rapidly, and she succumbed to hypovolemic shock before further confirmatory tests could be completed.

Why This Case Matters: Recognizing the Atypical

This case underscores the critical importance of considering rare diagnoses, like APL, in patients presenting with PPH, especially when typical causes have been ruled out or treatments are ineffective. While APL is rare during pregnancy, its aggressive nature necessitates prompt diagnosis and treatment.

The presence of disseminated intravascular coagulation (DIC) in PPH should raise suspicion for APL. Key indicators include: reduced platelet count, increased prothrombin time, activated partial thromboplastin time, reduced serum fibrinogen level, increased D-dimer level.

A simple peripheral blood smear can be life-saving. Early recognition of abnormal promyelocytes, followed by confirmatory bone marrow aspiration and molecular testing, allows for timely initiation of all-trans retinoic acid (ATRA) therapy, the standard treatment for APL, significantly improving patient outcomes.

About this Article -

This article was crafted using a human-AI hybrid and collaborative approach. AI assisted our team with initial drafting, research insights, identifying key questions, and image generation. Our human editors guided topic selection, defined the angle, structured the content, ensured factual accuracy and relevance, refined the tone, and conducted thorough editing to deliver helpful, high-quality information.See our About page for more information.

This article is based on research published under:

DOI-LINK: 10.5045/br.2013.48.4.299, Alternate LINK

Title: Acute Promyelocytic Leukemia With An Unusual Presentation Of Secondary Postpartum Hemorrhage

Subject: Hematology

Journal: Blood Research

Publisher: The Korean Society of Hematology

Authors: Sunita Sharma, Mukta Pujani, Narender Tejwani

Published: 2013-01-01

Everything You Need To Know

1

What is Acute promyelocytic leukemia (APL), and how does it relate to the described condition?

Acute promyelocytic leukemia (APL) is a rare and aggressive form of leukemia, a type of acute myeloid leukemia (AML). It's characterized by a specific genetic abnormality, the t(15;17) chromosomal translocation. This means there's a rearrangement of genetic material between chromosomes 15 and 17. This genetic change leads to the uncontrolled production of abnormal promyelocytes, which are immature white blood cells. In the context of postpartum hemorrhage (PPH), APL is significant because it can present with similar symptoms, making it a diagnostic challenge. Its implications are severe because if left untreated, APL progresses rapidly, as seen in the case, emphasizing the need for swift recognition and treatment.

2

What is secondary postpartum hemorrhage (PPH), and what are the implications of APL presenting with similar symptoms?

Secondary postpartum hemorrhage (PPH) is excessive bleeding that occurs after the first 24 hours following childbirth. While many factors can cause it, such as retained products of conception, uterine atony, or tears, the bleeding can also be caused by rarer conditions such as APL. This case demonstrates how APL can mimic common postpartum complications. Initial treatments for typical causes may fail. The recognition of unusual bleeding patterns or lack of response to standard treatments warrants a thorough investigation, including considering hematological disorders like APL.

3

What specific blood test results indicated a problem in the described case?

In the case highlighted, the initial blood tests revealed a hemoglobin level of 8.8 g/dL, indicating anemia, a low platelet count of 44 × 109/L (thrombocytopenia), and an abnormal leukocyte count, with 2% blasts and 65% promyelocytes. These are immature white blood cells that are not functioning properly. A peripheral blood smear confirmed the presence of abnormal promyelocytes, including some containing characteristic “faggot cells” (bundles of Auer rods). These findings, along with the patient's clinical presentation of excessive bleeding, raised suspicion for a hematological malignancy, specifically suggesting Acute promyelocytic leukemia (APL).

4

Why is a simple blood smear considered life-saving in this context?

A simple blood smear can be life-saving because it can quickly identify the presence of abnormal cells, such as promyelocytes and “faggot cells” (bundles of Auer rods). These cells are highly suggestive of Acute promyelocytic leukemia (APL). While it does not confirm the diagnosis, a blood smear provides critical information to prompt further investigations, such as bone marrow aspiration and genetic testing. In a case where a patient's condition is deteriorating rapidly, a blood smear can provide the earliest clue, as it helps to rule out other common causes of Postpartum hemorrhage (PPH) and to rapidly initiate treatment.

5

What are the most important diagnostic steps when considering Acute promyelocytic leukemia (APL) in a case of postpartum hemorrhage (PPH)?

The critical diagnostic steps include a comprehensive evaluation, especially when initial treatments for postpartum hemorrhage (PPH) fail. This includes a complete blood count (CBC), a peripheral blood smear, and possibly a bone marrow aspiration and biopsy to confirm the diagnosis of Acute promyelocytic leukemia (APL). Further tests include cytogenetic analysis to identify the t(15;17) translocation, which is the hallmark of APL. These steps are vital for differentiating between common causes of PPH and rarer, life-threatening conditions. Prompt diagnosis is essential for initiating timely treatment, such as chemotherapy with all-trans retinoic acid (ATRA) and anthracycline-based chemotherapy, which can improve survival rates in APL.

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