A pathologist receives a patient sample for analysis. Cells in the sample are first labeled with fluorescent antibodies and then passed across a laser beam in a single file of particles. The light scatter and fluorescent intensity of the particles are plotted on a graph; this information is used to characterize the sample. This laboratory method would be most useful to establish the diagnosis of a patient with which of the following?
Q132
A 15-year-old boy is brought to the physician because his urine has been pink since that morning. During the past 2 days, he has had a sore throat and difficulty swallowing. He also reports having a low-grade fever. He has no pain with urination or changes in urinary frequency. He has had 2 similar episodes involving a sore throat and pink urine over the past 2 years. His older sister has systemic lupus erythematosus. His temperature is 38.3°C (101°F), pulse is 76/min, and blood pressure is 120/80 mm Hg. Oral examination shows an erythematous pharynx and enlarged tonsils. The remainder of the examination shows no abnormalities. Laboratory studies show:
Leukocyte count 20,000/mm3
Serum
Urea nitrogen 8 mg/dL
Creatinine 1.4 mg/dL
Urine
Blood 3+
Protein 1+
RBC 15–17/hpf with dysmorphic features
RBC casts numerous
Ultrasound of both kidneys shows no abnormalities. A renal biopsy is most likely to show which of the following findings?
Q133
A 53-year-old man is brought to the emergency department by his wife because of a 1-day history of headache, blurry vision, and confusion. His wife also says that he hasn't urinated in the past 24 hours. Despite appropriate measures, the patient dies shortly after admission. A photomicrograph of a section of the kidney obtained at autopsy is shown. Which of the following is the most likely explanation for the findings indicated by the arrow?
Q134
A 17-year-old is brought to his primary care provider by his mother. They are concerned that his acne is getting worse and may leave scars. They have tried several over the counter products without success. He is otherwise healthy and without complaint. The boy was born at 39 weeks gestation via spontaneous vaginal delivery. He is up to date on all vaccines with some delay in reaching developmental milestones, especially in language development. He is having trouble with school and has a history of detentions for misbehavior and acting out. On examination, he stands 6 ft 3 inches tall, considerably taller than either parent or other family members with inflamed pustular acne on his face, shoulders, and chest. He is otherwise healthy. Which of the following karyotypes is most likely associated with this patient’s clinical features?
Q135
A 6-month-old male presents to the emergency department with his parents after his three-year-old brother hit him on the arm with a toy truck. His parents are concerned that the minor trauma caused an unusual amount of bruising. The patient has otherwise been developing well and meeting all his milestones. His parents report that he sleeps throughout the night and has just started to experiment with solid food. The patient’s older brother is in good health, but the patient’s mother reports that some members of her family have an unknown blood disorder. On physical exam, the patient is agitated and difficult to soothe. He has 2-3 inches of ecchymoses and swelling on the lateral aspect of the left forearm. The patient has a neurological exam within normal limits and pale skin with blue irises. An ophthalmologic evaluation is deferred.
Which of the following is the best initial step?
Q136
A 69-year-old white man comes to the physician because of a 15-day history of fatigue and lower leg swelling. Over the past 8 months, he has had a 3.8-kg (8.3-lb) weight loss. He has smoked one pack of cigarettes daily for 48 years. Vital signs are within normal limits. He appears thin. Examination shows 2+ pretibial edema bilaterally. An x-ray of the chest shows a right upper lobe density. Laboratory studies show:
Hemoglobin 11.3 g/dL
Leukocyte count 8600/mm3
Platelet count 140,000/mm3
Serum
Urea nitrogen 25 mg/dL
Glucose 79 mg/dL
Creatinine 1.7 mg/dL
Albumin 1.6 mg/dL
Total cholesterol 479 mg/dL
Urine
Blood negative
Glucose negative
Protein 4+
WBC 0–1/hpf
Fatty casts numerous
Light microscopic examination of a kidney biopsy reveals thickening of glomerular capillary loops and the basal membrane. Which of the following is the most likely diagnosis?
Q137
A 6-year-old boy presents to your office with hematuria. Two weeks ago the patient had symptoms of a sore throat and fever. Although physical exam is unremarkable, laboratory results show a decreased serum C3 level and an elevated anti-DNAse B titer. Which of the following would you most expect to see on renal biopsy?
Q138
A 50-year-old man presents to a clinic with oliguria. Four weeks ago, he had a kidney transplant. Postoperative follow-up was normal. He is currently on cyclosporine and admits that sometimes he forgets to take his medication. On physical examination, the vital signs include: temperature 37.1°C (98.8°F), blood pressure 165/110 mm Hg, heart rate 80/min, and respiratory rate 16/min. There is mild tenderness on renal palpation. His serum creatinine level is 4 mg/dL, well above his baseline level after the transplant. Which of the following best describes the histological finding if a biopsy is taken from the transplanted kidney?
Q139
A 22-year-old woman presents to her primary care physician complaining of a red, itchy rash on her elbows and shoulders for 2 months. She has no history of medical problems, and review of systems is positive only for occasional loose stools. She is appropriately prescribed dapsone, which relieves the rash within hours. What is the diagnosis?
Q140
A 10-year-old male presents with his mother with multiple complaints. A few weeks ago, he had a sore throat for several days that improved without specific therapy. Additionally, over the past several days he has experienced pain in his ankles and wrists and, more recently, his left knee. His mother also noted several bumps on both of his elbows, and he has also had some pain in his center of his chest. He thinks the pain is better when he leans forward. On physical examination, he is noted to be mildly febrile, and a pericardial friction rub is auscultated. Which of the following histopathologic findings is most likely associated with this patient's condition?
Systemic Pathology US Medical PG Practice Questions and MCQs
Question 131: A pathologist receives a patient sample for analysis. Cells in the sample are first labeled with fluorescent antibodies and then passed across a laser beam in a single file of particles. The light scatter and fluorescent intensity of the particles are plotted on a graph; this information is used to characterize the sample. This laboratory method would be most useful to establish the diagnosis of a patient with which of the following?
A. Ventricular septal defect and facial dysmorphism with low T-lymphocyte count
B. Painless generalized lymphadenopathy with monomorphic cells and interspersed benign histiocytes on histology
C. Pancytopenia and deep vein thrombosis with intermittent hemoglobinuria (Correct Answer)
D. Multiple opportunistic infections with decreased CD4 counts
E. Vesicular lesions with dermatomal distribution and dendritic corneal ulcers
Explanation: ***Pancytopenia and deep vein thrombosis with intermittent hemoglobinuria***
- The described laboratory method is **flow cytometry**, which is the **gold standard for diagnosing paroxysmal nocturnal hemoglobinuria (PNH)** by detecting the absence of **CD55** and **CD59** on red blood cells due to impaired GPI anchor synthesis.
- PNH classically presents with **pancytopenia**, **hemolytic anemia** (leading to hemoglobinuria), and a high risk of **thrombosis** (e.g., deep vein thrombosis).
*Ventricular septal defect and facial dysmorphism with low T-lymphocyte count*
- This clinical picture suggests **DiGeorge syndrome**, which involves a developmental defect of the **third and fourth pharyngeal pouches**, leading to thymic hypoplasia and **T-cell deficiency**.
- While flow cytometry is used to quantify T-lymphocyte subsets (e.g., CD3, CD4, CD8), the primary method for diagnosing DiGeorge syndrome is **fluorescent in situ hybridization (FISH)** for a **22q11 deletion**, making it less ideal for flow cytometry diagnosis.
*Painless generalized lymphadenopathy with monomorphic cells and interspersed benign histiocytes on histology*
- This description with "**monomorphic cells**" is more consistent with certain **non-Hodgkin lymphomas** (e.g., Burkitt lymphoma) rather than Hodgkin lymphoma, which typically shows a **polymorphic** cellular infiltrate.
- While flow cytometry can be useful in characterizing lymphomas by identifying cell surface markers, the diagnosis is primarily established by **lymph node biopsy and histopathology** with **immunohistochemistry**, making flow cytometry a supplementary rather than primary diagnostic tool.
*Multiple opportunistic infections with decreased CD4 counts*
- This presentation is highly suggestive of **HIV infection leading to AIDS**. The "decreased CD4 counts" are a key diagnostic and prognostic marker.
- While flow cytometry is used to **monitor CD4 cell counts** in HIV patients, the initial diagnosis of HIV is established via **antibody/antigen combination tests** and confirmed by **Western blot** or **PCR for viral load**, not by flow cytometry.
*Vesicular lesions with dermatomal distribution and dendritic corneal ulcers*
- This clinical presentation points to **herpes zoster ophthalmicus** (shingles affecting the eye due to **varicella-zoster virus** reactivation).
- Diagnosis is primarily **clinical** based on the characteristic rash and eye findings, although **PCR** of vesicular fluid can confirm VZV infection. Flow cytometry has no role in this diagnosis.
Question 132: A 15-year-old boy is brought to the physician because his urine has been pink since that morning. During the past 2 days, he has had a sore throat and difficulty swallowing. He also reports having a low-grade fever. He has no pain with urination or changes in urinary frequency. He has had 2 similar episodes involving a sore throat and pink urine over the past 2 years. His older sister has systemic lupus erythematosus. His temperature is 38.3°C (101°F), pulse is 76/min, and blood pressure is 120/80 mm Hg. Oral examination shows an erythematous pharynx and enlarged tonsils. The remainder of the examination shows no abnormalities. Laboratory studies show:
Leukocyte count 20,000/mm3
Serum
Urea nitrogen 8 mg/dL
Creatinine 1.4 mg/dL
Urine
Blood 3+
Protein 1+
RBC 15–17/hpf with dysmorphic features
RBC casts numerous
Ultrasound of both kidneys shows no abnormalities. A renal biopsy is most likely to show which of the following findings?
A. Splitting of the glomerular basement membrane
B. Capillary wire looping
C. IgA mesangial deposition (Correct Answer)
D. Crescents of fibrin and plasma proteins
E. Granular deposits of IgG, IgM, and C3 complement
Explanation: ***IgA mesangial deposition***
- The patient's presentation with **recurrent episodes of gross hematuria (pink urine) occurring simultaneously with pharyngitis** is highly suggestive of **IgA nephropathy (Berger's disease)**.
- **IgA mesangial deposition** is the hallmark finding on renal biopsy for IgA nephropathy, which is an immune complex-mediated glomerulonephritis.
- The **synpharyngitic** timing (hematuria concurrent with infection, not delayed) and **recurrent nature** are classic for IgA nephropathy.
*Splitting of the glomerular basement membrane*
- This finding is characteristic of **Alport syndrome**, a hereditary nephropathy associated with nerve deafness and ocular abnormalities, which are not present in this patient.
- While Alport can cause recurrent hematuria, the history of recurrent gross hematuria following pharyngitis points away from Alport syndrome.
*Capillary wire looping*
- **Capillary wire looping** refers to thickening of the glomerular capillary walls due to subendothelial immune deposits and is a classic feature of **diffuse proliferative glomerulonephritis**, often seen in **lupus nephritis**.
- Although the sister has lupus, the patient's symptoms and rapid resolution of previous episodes are less typical for acute lupus nephritis.
*Crescents of fibrin and plasma proteins*
- **Crescent formation** in Bowman's capsule is characteristic of **rapidly progressive glomerulonephritis (RPGN)**, indicating severe glomerular injury.
- While RPGN can present with hematuria, the patient has a history of recurrent, self-limiting episodes, which is not typical for the rapidly progressive and often severe course of crescentic glomerulonephritis.
*Granular deposits of IgG, IgM, and C3 complement*
- This pattern of immune deposits is typical of **post-streptococcal glomerulonephritis (PSGN)**, which also presents with hematuria following infection.
- However, PSGN typically has a **latent period of 1-3 weeks** between infection and renal symptoms, and **low C3 complement levels**, which are not seen in this patient; the quick onset and recurring episodes are more consistent with IgA nephropathy.
Question 133: A 53-year-old man is brought to the emergency department by his wife because of a 1-day history of headache, blurry vision, and confusion. His wife also says that he hasn't urinated in the past 24 hours. Despite appropriate measures, the patient dies shortly after admission. A photomicrograph of a section of the kidney obtained at autopsy is shown. Which of the following is the most likely explanation for the findings indicated by the arrow?
A. Mycotic aneurysm
B. Atherosclerotic plaque rupture
C. Necrotizing vasculitis
D. Chronic hyperglycemia
E. Severe hypertension (Correct Answer)
Explanation: ***Severe hypertension***
- The image likely shows **hyperplastic arteriolosclerosis** ("onion-skinning") or **fibrinoid necrosis** of arterioles, which are characteristic pathological changes seen in severe, **malignant hypertension**.
- This type of severe vascular damage explains the rapid onset of headache, blurry vision (hypertensive retinopathy), confusion (hypertensive encephalopathy), and acute kidney injury (lack of urination) due to widespread microvascular ischemia and organ dysfunction.
*Mycotic aneurysm*
- A **mycotic aneurysm** is an infected aneurysm, typically caused by bacterial endocarditis or other sources of bacteremia.
- While it can lead to stroke or organ ischemia, the histological image features are not consistent with an infectious process or an aneurysm, but rather with diffuse microvascular injury.
*Atherosclerotic plaque rupture*
- **Atherosclerotic plaque rupture** causes acute ischemic events by forming a thrombus on the ruptured plaque, typically in larger arteries.
- The presented clinical picture and histological findings (likely small arteriolar damage) are not typical of large vessel atherosclerosis.
*Necrotizing vasculitis*
- **Necrotizing vasculitis** involves inflammation and necrosis of vessel walls, which can lead to organ damage, but it's typically an autoimmune or inflammatory process.
- Although there may be some vascular necrosis in malignant hypertension (fibrinoid necrosis), the primary driver here is the sustained high pressure rather than a specific vasculitic syndrome.
*Chronic hyperglycemia*
- **Chronic hyperglycemia** leads to diabetic microangiopathy, characterized by **hyaline arteriolosclerosis** (protein deposition in vessel walls) and capillary basement membrane thickening.
- These changes develop slowly over years and would not explain the acute, severe presentation described, nor do they typically present with the "onion-skinning" appearance of severe hypertension.
Question 134: A 17-year-old is brought to his primary care provider by his mother. They are concerned that his acne is getting worse and may leave scars. They have tried several over the counter products without success. He is otherwise healthy and without complaint. The boy was born at 39 weeks gestation via spontaneous vaginal delivery. He is up to date on all vaccines with some delay in reaching developmental milestones, especially in language development. He is having trouble with school and has a history of detentions for misbehavior and acting out. On examination, he stands 6 ft 3 inches tall, considerably taller than either parent or other family members with inflamed pustular acne on his face, shoulders, and chest. He is otherwise healthy. Which of the following karyotypes is most likely associated with this patient’s clinical features?
A. 46 XY
B. 47 XYY (Correct Answer)
C. 45 X0
D. 47, XY, +21
E. 47 XXY
Explanation: ***47 XYY***
- The patient's **tall stature**, **developmental delays** (especially language), and **behavioral problems** are features that can be associated with **47, XYY syndrome**.
- Males with XYY syndrome are typically **taller than average** and may have **mild learning difficulties**, particularly with language and speech development.
- **Behavioral issues** such as impulsivity and attention problems have been reported, though the historical association with aggressive behavior has been largely debunked.
- While severe acne is mentioned in some older literature, it is not a well-established or consistent feature of XYY syndrome.
*46 XY*
- This is a **normal male karyotype** and would not explain the patient's constellation of symptoms, including exceptionally tall stature (significantly above family baseline) combined with developmental delays and behavioral issues.
- While a typical 46 XY individual can have acne or be tall, the combination and severity described are atypical for a normal karyotype.
*45 X0*
- This karyotype describes **Turner syndrome**, which affects females and is characterized by **short stature**, **ovarian dysfunction**, and specific physical features, none of which are present in this male patient.
- It's also incorrect because the patient is phenotypically male.
*47, XY, +21*
- This karyotype represents **Down syndrome**, characterized by specific **facial features**, **intellectual disability**, and various health problems, but not the exceptionally tall stature seen in this patient.
- The developmental delays in Down syndrome are also typically more generalized and severe than just language and behavioral issues.
*47 XXY*
- This karyotype describes **Klinefelter syndrome**, a condition found in males characterized by **tall stature**, **hypogonadism**, and often **gynecomastia**.
- While it can manifest with tall stature and some learning difficulties, **hypogonadism** would be expected on examination, which is not described in this patient.
- The clinical picture of isolated tall stature with developmental and behavioral issues without signs of hypogonadism is more consistent with XYY syndrome.
Question 135: A 6-month-old male presents to the emergency department with his parents after his three-year-old brother hit him on the arm with a toy truck. His parents are concerned that the minor trauma caused an unusual amount of bruising. The patient has otherwise been developing well and meeting all his milestones. His parents report that he sleeps throughout the night and has just started to experiment with solid food. The patient’s older brother is in good health, but the patient’s mother reports that some members of her family have an unknown blood disorder. On physical exam, the patient is agitated and difficult to soothe. He has 2-3 inches of ecchymoses and swelling on the lateral aspect of the left forearm. The patient has a neurological exam within normal limits and pale skin with blue irises. An ophthalmologic evaluation is deferred.
Which of the following is the best initial step?
A. Genetic testing
B. Complete blood count and coagulation panel (Correct Answer)
C. Ensure the child's safety and alert the police
D. Peripheral blood smear
E. Hemoglobin electrophoresis
Explanation: ***Complete blood count and coagulation panel***
- The unusual amount of **bruising** after minor trauma, along with a family history of an unknown blood disorder, strongly suggests a potential **bleeding disorder**. A **CBC** and a **coagulation panel** (PT, aPTT, fibrinogen) are essential initial steps to evaluate for abnormalities in platelets, clotting factors, or other hematologic conditions.
- These tests can help narrow down the differential diagnosis between **platelet dysfunction**, **coagulopathies** (like hemophilia or von Willebrand disease), or other less common bleeding disorders, guiding further specific investigations.
- **Important consideration:** The presence of **blue sclera** (described as "blue irises") raises concern for **osteogenesis imperfecta (OI)**, a connective tissue disorder causing bone fragility. However, initial hematologic screening is still appropriate given the family history of blood disorder and presentation of excessive bruising. If coagulation studies are normal, imaging and further workup for OI would be indicated.
*Genetic testing*
- While a genetic component is plausible given the patient's family history and clinical presentation (blue sclera may suggest osteogenesis imperfecta), **genetic testing** is typically performed *after* initial laboratory workup has identified a specific type of bleeding or inherited disorder.
- Starting with genetic testing without basic hematologic parameters is not the most efficient or cost-effective initial diagnostic approach.
*Ensure the child's safety and alert the police*
- While child abuse should always be considered in cases of unexplained or excessive bruising, the presence of a **family history of a blood disorder** and the **blue sclera** (suggesting possible osteogenesis imperfecta) make **medical causes** more immediate concerns for initial investigation.
- Pursuing a medical workup first often clarifies whether abuse is the primary explanation, although child protective services should be notified if suspicion remains high after medical evaluation.
*Peripheral blood smear*
- A **peripheral blood smear** provides information on red blood cell morphology, platelet size and number, and white blood cell differential. While useful in assessing for some hematologic conditions, it is often performed *after* a CBC has indicated abnormalities or in conjunction with specialized testing.
- It would not be the *best initial step* as it doesn't directly assess clotting factor function, which is critical in evaluating significant bruising severity.
*Hemoglobin electrophoresis*
- **Hemoglobin electrophoresis** is used to diagnose **hemoglobinopathies** like sickle cell anemia or thalassemia.
- The patient's symptoms (easy bruising) are not characteristic of hemoglobinopathies, and while he has pale skin, this test would not be the initial step to investigate a bleeding disorder.
Question 136: A 69-year-old white man comes to the physician because of a 15-day history of fatigue and lower leg swelling. Over the past 8 months, he has had a 3.8-kg (8.3-lb) weight loss. He has smoked one pack of cigarettes daily for 48 years. Vital signs are within normal limits. He appears thin. Examination shows 2+ pretibial edema bilaterally. An x-ray of the chest shows a right upper lobe density. Laboratory studies show:
Hemoglobin 11.3 g/dL
Leukocyte count 8600/mm3
Platelet count 140,000/mm3
Serum
Urea nitrogen 25 mg/dL
Glucose 79 mg/dL
Creatinine 1.7 mg/dL
Albumin 1.6 mg/dL
Total cholesterol 479 mg/dL
Urine
Blood negative
Glucose negative
Protein 4+
WBC 0–1/hpf
Fatty casts numerous
Light microscopic examination of a kidney biopsy reveals thickening of glomerular capillary loops and the basal membrane. Which of the following is the most likely diagnosis?
A. Rapidly progressive glomerulonephritis
B. Granulomatosis with polyangiitis
C. Membranous nephropathy (Correct Answer)
D. Focal segmental glomerulosclerosis
E. Membranoproliferative glomerulonephritis
Explanation: ***Membranous nephropathy***
- The patient's presentation with **nephrotic syndrome** (edema, severe proteinuria 4+, hypoalbuminemia, hyperlipidemia, and fatigue) in an older adult, along with a **right upper lobe density** concerning for malignancy, strongly suggests secondary membranous nephropathy.
- The renal biopsy finding of **thickening of glomerular capillary loops and the basal membrane** is a hallmark of membranous nephropathy.
*Rapidly progressive glomerulonephritis*
- Characterized by a **rapid decline in renal function** over days to weeks, often with oliguria and symptoms of severe systemic illness.
- While proteinuria can be present, the classic biopsy finding is **crescent formation**, which is not described here.
*Granulomatosis with polyangiitis*
- This is a systemic vasculitis often involving the **upper and lower respiratory tracts** and kidneys, commonly presenting with **hemoptysis, sinusitis, and glomerulonephritis**.
- While a lung density is present, the absence of **hemoptysis** and the specific biopsy findings of membranous nephropathy make it less likely.
*Focal segmental glomerulosclerosis*
- This typically causes nephrotic syndrome but is characterized by **segmental sclerosis of glomeruli** on biopsy.
- While it can be idiopathic or secondary, the specific biopsy description and association with a potential malignancy point away from FSGS.
*Membranoproliferative glomerulonephritis*
- This condition presents with features of both **nephrotic and nephritic syndromes** and is characterized by a "tram-track" appearance on biopsy due to mesangial cell proliferation and basement membrane splitting.
- This specific microscopic finding is not mentioned in the patient's biopsy report.
Question 137: A 6-year-old boy presents to your office with hematuria. Two weeks ago the patient had symptoms of a sore throat and fever. Although physical exam is unremarkable, laboratory results show a decreased serum C3 level and an elevated anti-DNAse B titer. Which of the following would you most expect to see on renal biopsy?
A. Immune complex deposits with a "spike and dome" appearance on electron microscopy
B. Large, hypercellular glomeruli on light microscopy (Correct Answer)
C. Wirelooping and hyaline thrombi on light microscopy
D. Polyclonal IgA deposition on immunofluorescence
E. Antibodies to GBM resulting in a linear immunofluorescence pattern
Explanation: ***Large, hypercellular glomeruli on light microscopy***
- The clinical presentation, including recent **sore throat**, **hematuria**, decreased **C3**, and elevated **anti-DNAse B titer**, is highly suggestive of **post-streptococcal glomerulonephritis (PSGN)**.
- On light microscopy, PSGN characteristically shows **diffuse proliferative glomerulonephritis** with **enlarged, hypercellular glomeruli** due to inflammatory cell infiltration and proliferation of intrinsic glomerular cells.
*Immune complex deposits with a "spike and dome" appearance on electron microscopy*
- This "spike and dome" appearance refers to **subepithelial deposits** with new basement membrane formation, which is characteristic of **membranous nephropathy**, not PSGN.
- Membranous nephropathy typically presents with **nephrotic syndrome** in adults, which is different from the child's presentation here.
*Wirelooping and hyaline thrombi on light microscopy*
- These features, particularly **"wire-loop" lesions** and **hyaline thrombi**, suggest severe **lupus nephritis**, a manifestation of **systemic lupus erythematosus**.
- Lupus nephritis often presents with a range of kidney pathologies and systemic symptoms not seen in this patient.
*Polyclonal IgA deposition on immunofluorescence*
- **IgA deposition** in the mesangium is the hallmark of **IgA nephropathy (Berger's disease)**, which typically presents with recurrent hematuria, often after an upper respiratory infection.
- While there is hematuria and a recent infection, the decreased C3 and elevated anti-DNAse B titer point away from IgA nephropathy.
*Antibodies to GBM resulting in a linear immunofluorescence pattern*
- A **linear pattern** of IgG deposits along the glomerular basement membrane (GBM) is characteristic of **Goodpasture syndrome (anti-GBM disease)**.
- This condition involves antibodies specifically targeting the GBM and often presents with rapidly progressive glomerulonephritis and pulmonary hemorrhage, which are not described here.
Question 138: A 50-year-old man presents to a clinic with oliguria. Four weeks ago, he had a kidney transplant. Postoperative follow-up was normal. He is currently on cyclosporine and admits that sometimes he forgets to take his medication. On physical examination, the vital signs include: temperature 37.1°C (98.8°F), blood pressure 165/110 mm Hg, heart rate 80/min, and respiratory rate 16/min. There is mild tenderness on renal palpation. His serum creatinine level is 4 mg/dL, well above his baseline level after the transplant. Which of the following best describes the histological finding if a biopsy is taken from the transplanted kidney?
A. Lymphocytic infiltration of graft vessels and endothelial damage (Correct Answer)
B. Thrombosis and occlusion of vessels
C. Atherosclerosis on angiography
D. Necrosis with granulation tissue
E. Thickening of blood vessels, fibrosis of graft vessels, and parenchymal atrophy
Explanation: ***Lymphocytic infiltration of graft vessels and endothelial damage***
- The patient's presentation with **oliguria**, elevated **creatinine**, and **hypertension** following a recent kidney transplant, especially with a history of non-adherence to **cyclosporine** (an immunosuppressant), strongly indicates **acute rejection**.
- Histologically, acute rejection is characterized by **lymphocytic infiltration** of the graft vessels (often referred to as **vasculitis** or **endotheliitis**) and associated **endothelial damage**.
*Thrombosis and occlusion of vessels*
- This finding is more characteristic of **hyperacute rejection**, which typically occurs within minutes to hours of transplantation, not weeks later.
- Hyperacute rejection is mediated by **pre-formed antibodies** and leads to severe, rapid graft failure due to widespread intravascular thrombosis.
*Atherosclerosis on angiography*
- While post-transplant patients can develop accelerated atherosclerosis (a form of **chronic rejection**), it is typically a long-term complication developing months to years after transplantation.
- The acute presentation with rapid creatinine elevation is not typical for primary atherosclerosis.
*Necrosis with granulation tissue*
- **Necrosis** with **granulation tissue** is a general healing response to significant tissue injury or inflammation.
- While some cellular necrosis can occur in severe rejection, it's not the defining feature, and granulation tissue indicates a more prolonged, subacute process rather than the primary histological hallmark of acute rejection.
*Thickening of blood vessels, fibrosis of graft vessels, and parenchymal atrophy*
- These are classic features of **chronic rejection**, which manifests months to years after transplantation as a gradual decline in graft function.
- **Chronic rejection** involves progressive damage leading to vasculopathy, interstitial fibrosis, and tubular atrophy, rather than the acute inflammatory cellular infiltrate seen here.
Question 139: A 22-year-old woman presents to her primary care physician complaining of a red, itchy rash on her elbows and shoulders for 2 months. She has no history of medical problems, and review of systems is positive only for occasional loose stools. She is appropriately prescribed dapsone, which relieves the rash within hours. What is the diagnosis?
A. Porphyria cutanea tarda
B. Candida intertrigo
C. Leprosy
D. Dermatitis herpetiformis (Correct Answer)
E. Systemic lupus erythematosus
Explanation: ***Dermatitis herpetiformis***
- The combination of a **red, itchy rash** on the **elbows and shoulders**, occasional **loose stools**, and a rapid response to **dapsone** is highly characteristic of dermatitis herpetiformis.
- This condition is strongly associated with **celiac disease** (explaining the loose stools) and is characterized by IgA deposits in the dermal papillae, which are targeted by dapsone.
*Porphyria cutanea tarda*
- This condition presents with **photosensitive blistering lesions**, often on sun-exposed areas, and is associated with **liver disease** and iron overload, which are not described here.
- While skin fragility and hypertrichosis can occur, the itching and dapsone response are not typical features.
*Candida intertrigo*
- This is a **fungal infection** typically found in **skin folds** (e.g., groin, axilla, inframammary folds) and presents with a beefy red rash with satellite lesions.
- It would not typically affect the elbows and shoulders in this manner, nor would it respond rapidly to dapsone.
*Leprosy*
- Leprosy presents with **hypopigmented or erythematous skin lesions** with **sensory loss** and nerve involvement.
- The symptoms described (itchy rash, specific location, quick dapsone response for itch) are not consistent with leprosy.
*Systemic lupus erythematosus*
- SLE can present with various skin manifestations, including a **malar rash** or discoid lesions, but a highly itchy rash on the elbows and shoulders rapidly responding to dapsone is not typical.
- SLE is a **multi-system autoimmune disease** with other characteristic symptoms (e.g., arthralgia, fatigue, serositis) and laboratory findings that are not mentioned.
Question 140: A 10-year-old male presents with his mother with multiple complaints. A few weeks ago, he had a sore throat for several days that improved without specific therapy. Additionally, over the past several days he has experienced pain in his ankles and wrists and, more recently, his left knee. His mother also noted several bumps on both of his elbows, and he has also had some pain in his center of his chest. He thinks the pain is better when he leans forward. On physical examination, he is noted to be mildly febrile, and a pericardial friction rub is auscultated. Which of the following histopathologic findings is most likely associated with this patient's condition?
A. Interstitial myocardial granulomas containing plump macrophages with nuclei incorporating central wavy ribbons of chromatin (Correct Answer)
B. Sterile vegetations on both the ventricular and atrial aspects of the mitral valve, a proliferative glomerulonephritis, and serum anti-dsDNA and anti-Sm positivity
C. Needle-shaped, negatively birefringent crystal deposits surrounded by palisading histiocytes in the synovial fluid of an affected joint
D. Plasmodium falciparum ring forms in red blood cells on peripheral blood smear
E. Atypical lymphocytes noted on peripheral blood smear with an initial positive heterophil antibody test
Explanation: ***Interstitial myocardial granulomas containing plump macrophages with nuclei incorporating central wavy ribbons of chromatin***
- The clinical presentation, including a recent **sore throat**, **migratory polyarthritis**, **subcutaneous nodules** (bumps on elbows), **pericardial pain** relieved by leaning forward, and **pericardial friction rub**, is highly suggestive of **acute rheumatic fever (ARF)**.
- **Aschoff bodies**, which are **interstitial myocardial granulomas** containing **Anitschkow cells** (plump macrophages with centrally wavy chromatin, often described as "caterpillar cells"), are the **pathognomonic histopathologic finding** for ARF.
*Sterile vegetations on both the ventricular and atrial aspects of the mitral valve, a proliferative glomerulonephritis, and serum anti-dsDNA and anti-Sm positivity*
- This description points to **Libman-Sacks endocarditis** in the context of **systemic lupus erythematosus (SLE)**, characterized by **sterile vegetations** on both sides of the valve and associated with **lupus nephritis** and specific **autoantibodies (anti-dsDNA, anti-Sm)**.
- While ARF can cause endocarditis, the vegetations are typically on the atrial side and not usually associated with the described glomerulonephritis or autoantibody profile.
*Needle-shaped, negatively birefringent crystal deposits surrounded by palisading histiocytes in the synovial fluid of an affected joint*
- This describes the characteristic findings in **gout**, where **monosodium urate crystals** accumulate in joints, causing inflammatory arthritis.
- The patient's symptoms of migratory polyarthritis and pericarditis are not typical for gout, and gout is rare in children.
*Plasmodium falciparum ring forms in red blood cells on peripheral blood smear*
- This is the diagnostic finding for **malaria**, specifically infection with *Plasmodium falciparum*.
- The patient's symptoms, while including fever, do not suggest malaria, and signs such as arthritis and pericarditis are not primary features of malaria.
*Atypical lymphocytes noted on peripheral blood smear with an initial positive heterophil antibody test*
- This describes the classic laboratory findings for **infectious mononucleosis**, typically caused by the **Epstein-Barr virus (EBV)**.
- While a sore throat can precede mononucleosis, the severe migratory arthritis, subcutaneous nodules, and pericarditis are not characteristic features of this condition.