A 21-year-old Female with Acute Liver Failure and Hemolytic Anemia

Had an interesting case this month that my Blood Bank team was involved with. I've changed some of the information to protect her privacy.

A 21-year-old female presented with a 5 week history of fatigue, anorexia, and mild jaundice. She was previously healthy with no past medical history, no medications, and no drug use. The initial workup showed a normochromic, normocytic anemia (Hgb 11.5) and increased liver function tests and was negative for infectious mononucleosis and viral hepatitis. Just a few days after this basic workup her course worsened and she became more severely jaundiced and developed dark urine and right upper quadrant pain. She was admitted to a referring institution and the workup showed the following:

Afebrile and vital signs stable
Scleral icterus, jaundice, splenomegaly, and RUQ pain on exam

WBC: 13.5 H
Hgb: 6.5 L
Plt: 178
Creatinine: 2.2 H

ALT: 13
AST: 147 H
Alk phos: 17 L
Tbili: 42.9 H
Dbili: 12.6 H

INR: 3.1 H
Albumin: 2.3 L

LDH: 659 H
Haptoglobin: undetectable L
Retic: 11.79 H
Direct antiglobulin: neg.
Blood type: O+, Ab screen neg.

ANA: within normal limits
Serum pregnancy test: neg.

Does anyone have a diagnosis? I'll post it in a week with follow up photos in a new post and/or comment.

A 30-year-old man with an intracranial parafalcine mass

We received an unusual case on our neuropathology service this week. The specimen was a 4 cm frontal, extra-axial mass that was dural-based, abutting the falx cerebri. MRI showed intense enhancement with surrounding vasogenic edema and a small dural tail. There was only mild mass effect and diffusion weighted imaging (DWI) showed no diffusion restriction. Cerebral arteriography showed a hypervascular lesion. Left middle meningeal artery embolization was performed preoperatively. At craniotomy the tumor was liquid and necrotic-appearing and the excised specimen was sent to us for pathological examination. The histological appearance is depicted below (click on photos to get higher resolution):

Glassy, eosinophilic cytoplasm with inclusions

Atypical cytology with some prominent nucleoli (occasional mitoses were also identified [~1-2 per 10 high-powered fields])

Focal areas of necrosis and hemorrhage

MIB-1 (Ki-67) immunostain showing a 15-20% proliferation rate.

Additionally, a vimentin immunostain showed strong diffuse cytoplasmic positivity and an EMA immunostain showed positive membrane staining. Cytokeratin immunostain was negative. How would you diagnose this case?

What to read?

Pathology is one of the study-heavy specialties, along with others like Radiology and Radiation Oncology. The benefit of a "normal", "predictable" workday is offset by all the reading you must do on the side, over the weekend and holidays, and when family thinks that you've finished the crazy studying routine set forth in the formative years. My choice for tonight is one of the main Pathology journals, as shown above: Archives of Pathology and Laboratory Medicine. It has a resident-friendly format with, in addition to original research articles, teaching cases and a differential diagnosis approach to certain findings after imaging or microscopic examination.

Medical school certainly doesn't prepare a student for any one specialty. Of course it can't. But, surgeons may start residency having only held retractors for 10-hour long surgeries. Family physician residents may not have done simple office procedures. Emergency med residents may never have placed defibrillator pads on a live patient.

In Pathology, most of my fellow residents and I feel particularly unprepared to begin residency in this specialty. Yes, we get histology and some basic pathology during the first and second years of medical school. Perhaps we saw an autopsy or two. But we certainly didn't learn how to formulate a report for cases that we now see. Or do an autopsy mostly by ourselves and know how to pay particular attention to certain areas of the body in order to pick up key findings. For example, there are special, non-routine ways to check for pneumothorax or air embolism, if those are suspected as a cause of death. If not suspected clinically it may not be tested for and thus, not uncovered.

For Surgery, Family Practice, Internal Medicine...you are expected to be a somewhat-functioning resident as you step into the physician shoes on day 1. Hence all the training in learning to interview patients, write History and Physicals, check and interpret lab test results, and present cases to fellow colleagues on rounds or over the phone.

As for us, the Pathology, Radiology, Radiation Oncology residents out there, we generally found our field of choice by doing a rotation during medical school in our respective fields. But these were usually "litmus test" rotations so that we assured ourselves of choosing something in which we have genuine interest so we can happily practice for the rest of our career. Maybe we pick up a few useful skills, but nothing on the order of super-practical.

I am not bitter about the lack of preparation for Pathology. However, I think important improvements could be made in the way basic pathophysiology can be taught and integrated with clinical learning. One solution is the clinicopathological case (CPC) based conferences. They are of particular interest and relevance, I believe. They are the same format as those published weekly by the New England Journal of Medicine. Our teaching institution has similar conferences, but just once per month. At the Dartmouth-Hitchcock Medical Center in New Hampshire, there were weekly CPC conferences (M&M conferences). I love these types of presentations - integrations of research, pathology, clinical cases, and quality improvement. My vote: more, please!