HY USMLE Q #1035 – Pathology
Summary
TLDRThis educational video script discusses a pathology case of a 7-year-old boy with Burkitt lymphoma, characterized by a 'starry sky' appearance in histopathology due to tingible body macrophages engulfing apoptotic B cells. The script clarifies common misconceptions about the disease's presentation and emphasizes the importance of recognizing the 814 translocation associated with Burkitt lymphoma, contrasting it with other lymphomas and leukemias, such as follicular lymphoma and APL. It concludes with a call to action for viewers to support more content creation.
Takeaways
- 🔬 The pathology image described is indicative of Burkitt lymphoma, characterized by a 'starry sky' appearance due to the presence of basophilic background of B cells and pale, larger cells which are tingible body macrophages.
- 📚 Tingible body macrophages are known to engulf apoptotic B cells, which is an important histological feature to recognize in Burkitt lymphoma.
- 🧬 The t(8;14) translocation is associated with Burkitt lymphoma, involving the c-Myc gene, a transcription factor, and is a key genetic abnormality to know for exams.
- ❌ The script clarifies that t(14;18) translocation is incorrect for Burkitt lymphoma; instead, it is associated with follicular lymphoma and involves an anti-apoptotic molecule, BCL2.
- 📈 Follicular lymphoma often presents as a waxing and waning painless neck mass and is the most common indolent (slow-growing) non-Hodgkin lymphoma.
- 🧬 The t(15;17) translocation is associated with acute promyelocytic leukemia (APL), also known as AML type M3, and involves the PML-RARA gene fusion.
- 💊 All-trans retinoic acid is a treatment for APL, highlighting the importance of knowing therapeutic options for specific genetic abnormalities in leukemia.
- 🧬 The Philadelphia chromosome, or t(9;22) translocation, results in the BCR-ABL fusion protein and is associated with chronic myeloid leukemia (CML), a high-yield topic for medical exams.
- 📝 The script emphasizes the importance of understanding the genetic abnormalities and their clinical correlations for various types of lymphoma and leukemia, which are crucial for medical examinations.
Q & A
What is the most likely diagnosis for a 7-year-old boy with a one-month history of generalized Abdus, fatigue, and a 2 cm mass left of the umbilicus?
-The most likely diagnosis, based on the description and biopsy findings, is Burkitt lymphoma, which is characterized by a starry sky appearance with basophilic background of Burkitt B cells and tingible body macrophages.
What does the term 'starry sky' appearance refer to in the context of Burkitt lymphoma?
-The 'starry sky' appearance refers to the characteristic histological feature of Burkitt lymphoma where pale-staining macrophages are scattered among darker staining lymphoma cells, resembling stars in the sky.
What is the significance of tingible body macrophages in Burkitt lymphoma?
-Tingible body macrophages are significant because they indicate the presence of apoptosis within the tumor. These macrophages engulf and digest apoptotic Burkitt B cells, contributing to the 'starry sky' appearance.
What is the role of apoptosis in Burkitt lymphoma despite it being a hyper-proliferating cancer?
-Even though Burkitt lymphoma is a rapidly proliferating cancer, there is still cell turnover occurring, leading to apoptosis. This process is highlighted by the presence of tingible body macrophages that phagocytose apoptotic B cells.
What is the significance of the 814 translocation in Burkitt lymphoma?
-The 814 translocation is significant as it is almost always associated with Burkitt lymphoma and involves the c-Myc gene, a transcription factor that plays a role in the pathogenesis of the disease.
How does the presentation of Burkitt lymphoma differ from that of follicular lymphoma?
-Burkitt lymphoma often presents with abdominal masses, while follicular lymphoma typically presents as a waxing and waning painless neck mass over one to two years. Follicular lymphoma is also associated with a 14;18 translocation involving the BCL2 gene.
What is the treatment implication of the 15;17 translocation in acute promyelocytic leukemia (APL)?
-The 15;17 translocation in APL results in the fusion of the PML and RARA genes, leading to the production of a PML-RARA fusion protein. All-trans retinoic acid can be used as a treatment to differentiate the leukemic cells.
What is the Philadelphia chromosome associated with, and what is its clinical significance?
-The Philadelphia chromosome is associated with a 9;22 translocation, leading to the fusion of the BCR and ABL genes. This fusion protein, BCR-ABL, is a constitutively active tyrosine kinase, driving the pathogenesis of chronic myeloid leukemia (CML).
Why is it important to know the difference between the translocations associated with Burkitt lymphoma and follicular lymphoma?
-Knowing the difference between the translocations for Burkitt lymphoma (8;14) and follicular lymphoma (14;18) is important for accurate diagnosis and treatment planning, as they involve different genes and have distinct clinical presentations and outcomes.
What is the significance of the term 'indolent' when describing non-Hodgkin lymphomas?
-The term 'indolent' refers to a slow-growing, less aggressive form of non-Hodgkin lymphoma, such as follicular lymphoma. These types of lymphomas often have a more chronic course and may require different treatment approaches compared to more aggressive forms.
Outlines
🔬 Burkitt Lymphoma Pathology Discussion
The paragraph discusses a case of a 7-year-old boy with a one-month history of generalized Abdus, fatigue, and weight loss, leading to the discovery of a 2 cm mass in the abdomen. A biopsy of this mass reveals a 'Starry Sky' appearance, characteristic of Burkitt lymphoma. The discussion highlights the presence of basophilic (purple/blue) background of Burkitt lymphoma B cells and tingible body macrophages, which are known to engulf apoptotic B cells. The video script emphasizes the importance of recognizing the 814 translocation associated with Burkitt lymphoma and the c-Myc gene, which is a transcription factor. It contrasts Burkitt lymphoma with follicular lymphoma, which is associated with a 148;18 translocation and involves an anti-apoptotic molecule, BCL2. The script also touches upon other lymphomas and translocations, such as APL (acute promyelocytic leukemia) with a 1517 translocation and CML (chronic myeloid leukemia) with a 922 translocation involving the BCR-ABL fusion protein.
Mindmap
Keywords
💡Burkitt Lymphoma
💡Tingible Body Macrophages
💡Apoptosis
💡Translocation
💡Follicular Lymphoma
💡BCL2
💡Hodgkin Lymphoma
💡Acute Promyelocytic Leukemia (APL)
💡Philadelphia Chromosome
💡Indolent Lymphoma
💡African Boy with Jaw Lesion
Highlights
A seven-year-old boy presents with a one-month history of generalized Abdus, fatigue, and weight loss.
Vital signs are normal, and a palpable 2 cm mass is found on the left of the abdomen.
Biopsy of the mass reveals a starry sky appearance, characteristic of Burkitt lymphoma.
The presence of basophilic background with tingible body macrophages indicates Burkitt lymphoma.
Tingible body macrophages are known to phagocytose B cells undergoing apoptosis in Burkitt lymphoma.
Burkitt lymphoma is associated with an 814 translocation involving the c-Myc gene.
Follicular lymphoma is contrasted with Burkitt lymphoma, being associated with a 148;18 translocation and bcl2 gene.
Follicular lymphoma often presents as a waxing waning painless neck mass over one to two years.
Burkitt lymphoma can present with intraabdominal or jaw lesions, causing kyus accumulation.
Burkitt lymphoma is a high-grade, aggressive B-cell non-Hodgkin lymphoma.
Follicular lymphoma is the most common indolent (slow-growing) non-Hodgkin lymphoma.
Hodgkin lymphoma is characterized by the presence of Reed-Sternberg cells.
Acute promyelocytic leukemia (APL) is associated with a 15;17 translocation and the PML-RARA gene fusion.
APL can be treated with all-trans retinoic acid as well as standard AML therapy.
The Philadelphia chromosome, a 9;22 translocation, is associated with a BCR-ABL fusion protein in chronic myeloid leukemia (CML).
BCR-ABL is an oncogenic tyrosine kinase, a key factor in the development of CML.
Understanding the translocations and gene fusions is crucial for diagnosing and treating various types of lymphoma and leukemia.
Transcripts
how's it going guys past level question
pathology hysto step one seven-year-old
boy one month history generalized Abdus
Comfort fatigue lost five pounds during
this time vitals are normal de palpation
of the abdomen shows 2 cm Mass left of
them like is biopsy of the mass shown
question on most likely explanation for
finding so let's just hop through well
why don't we look at the hiso first so
very Buzzy easy image of the Starry Sky
appearance for Burk lymphoma so you have
basophilic which means purple/blue the
basophilic background of fitus B cells
okay and then you've got more pale
appearing larger cells these are
macrofagos and the US wants you to know
it's asked on one of the forms that
these are known as tingible not tangible
tingible body macrofagos and they have
an arrow pointing to one of the
macrofagos and the answer is apoptosis
so you need to know that these
macrophages will mop up SL AG oyos some
of the burket lymphoma B cells that are
undergoing apoptosis sounds a little
weird you say well it's a cancer it's
hyper proliferating but there is still
some turnover occurring and so these are
tingible body mafes they phagocytose B
cells undergoing apoptosis so I don't
really know what to tell you they want
you to know that so let's just hop to
the answers here Choice a 814
translocation wrong answer with
anti peptic molecule because the reason
it's wrong is because Choice B is the
answer burka is an 814 translocation
almost always with the c mik Gene which
is a transcription Factor okay not
dramatic it's past level as I said so
you do need to know in contrast that
actually before I even go to that bural
lymphoma students will often memorize it
as African boy with a jaw lesion but you
need to know that it can be
intraabdominal I've seen it on one of
the 2ck forms coincidentally where they
give you an intraabdominal lymphoma and
it's Burk it okay causing a um a kyus
accumulation kyus means lymph fluid so
kyus accumulation within the perinal
cavity in that question but you need to
know it can be intraabdominal or of the
jaw
so follicular lymphoma in contrast 148
18 translocation is going to be
anti-apoptotic molecule with
bcl2 okay so bcl2 anti-apoptotic
molecule folic Loma they might tell you
that there's a waxing waning painless
neck Mass over one to two years that's
how folicular lymphoma can present it's
the most common indolent which means
lazy non hodkin lymphoma okay so these
are all bkid and folicular non hodal
lymphomas lengthy disc discussion okay
hod can obviously read Sternberg cells
all right so let's just hop to the final
ones here 1517 translocation wrong
answers these are going to be
APL acute promy acidic leukemia AKA AML
type M3 and that's actually going to be
a fusion protein PML R Alpha Gene okay
they're not obsessed with the
translocation highest yield point is
that you're going to see a smear we have
our rods okay you need to know all trans
retinoic acid can be a treatment for APL
as well as just regular AML okay and
then finally 922 translocation wrong
answer this is going to be
fusion protein with Philadelphia
chromosome BCR abl this this is CML okay
chronic myologist AIA one the highest
yield conditions on us smla so BC a it's
a fusion protein that can be the answer
it can also be as the answer
oncogenic tyrosine kinas okay they want
you to know that it's a l discussion
regarding all these pathologies point is
wrong
answer you know the deal to make more
content like my stuff Channel appreciate
your time that's it
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