Problem based on juvenile-on-set diabetes mellitus

Question

Assignment task: Hematology Review

Instructions:

1. Please print this document (5 case studies). Read the cases carefully and answer the questions.

2. CBC units are given in Case Study A and are the same throughout the cases.

3. These are real patient case studies and they are meant to be challenging!!! You will need to use your notes, books and other resources for assistance in answering the questions.

4. To submit answer you will need to type them in using the assignment icon associated with these case studies. Short answers are preferable but you need to provide enough information for me to fully understand your answer. You will not get an immediate score. I have to manually grade this assignment. Be patient!

5. Submit the answers to the case study before due date. No extensions will be given. No late answers accepted.

6. Answers will be posted after all cases have been submitted.

Case Study A:

History: A 6-year-old male is admitted to the hospital for a work up for juvenile-on-set diabetes mellitus. He was also being treated for a macrocytic anemia.

Lab results were:

WBC 2.9 (x10⁹/L)

RBC 2.53 (x10¹²/L)

HGB 10.2 (g/dL)

HCT 29.3 (%)

MCV 116.0 fL

MCH 40.6 (pg)

MCHC 34.2 (%)

RDW 18.1 (%)

PLT 76 (x10⁹/L)

Retic 0.4%

Question 1: What is the most likely diagnosis based on the lab results above?

Additional testing:

Bone Marrow:

• Decreased M:E ratio
• 3% Blasts

Peripheral Blood Smear:

• Macrocytic RBCs
• Low platelet count
• Hyposegmented neutrophils

Treatment:

Patient was given vitamin B12 for 6 weeks- no change in the anemia status (patient did not respond to treatment)

Question 2: Does our patient have megaloblastic anemia? Why or why not?

Question 3: Looking at ALL the lab results again, what is another possible diagnosis for this patient?

Patient Outcome:  Patient was given supportive therapy for 4 years (packed red blood cells and platelets). Patient died of overwhelming infections

Case Study B:

History: A 44-year-old male was first seen in the VA hospital and then transferred to the city's larger hospital for re-evaluation. He was not responding to treatment that was prescribed.

Lab results were:

WBC 122.2

RBC 2.48

HGB 8.2

HCT 26.0

MCV 90.7

MCH 32.9

MCHC 36.3

RDW 14.3

PLT 27

Coagulation:

• Fibrinogen 67 mg/dl
• PT 19.3 sec
• INR 2.0
• PTT 39.5 sec
• FSP >40 µg/dl
• D-dimer 4250 NG/ML

Question 1: What do the above lab results indicate?

Additional testing

Peripheral Blood Smear Day 1:

• Several large WBCs with a large amount of nucleus, bi-lobed nucleus and hypogranular.
• These cells were reported as monocytes (monocytosis)

Peripheral Blood Smear Day 2:

On this day the MT performing the differential called the abnormal WBCs hypogranular promyelocytes.

Question 2: Based on the additional testing from Day 1, what is the best possible diagnosis?

Yet the results were different on Day 2, what is the best possible diagnosis based on the peripheral blood smear results from Day 2?

Patient Outcome: Patient died about 3 months later

Case Study C:

History: A 4-year-old male presents to his local pediatrician with irritability, loss of appetite and a distended abdomen. (Patient has a twin brother who is healthy and has not previous history of illness.) On physical exam the patient was febrile and irritable. The spleen was massively enlarged, liver was normal size and there was not lymphadenopathy.

Lab results were:

WBC 298.9

RBC 3.38

HGB 11.0

HCT 33.0

MCV 85.6

MCH 30.6

MCHC 33.2

RDW 17.4

PLT 366

Retic 1.4%

LAP Score: 6

• Philadelphia chromosome: Negative

Bone Marrow:

• Hypercellular marrow

Peripheral Blood Smear:

• All immature stages seen including bands, myelocytes, metamyelocytes, promyelocytes, blasts. Absolute monocytosis was aboserved.
• Kleihaur-Betke stain indicated that roughly 45% of RBCs contained HbF.

Question 1: What is the best diagnosis based on all the lab results? (Be specific this is a subgroup disease.)

Patient Outcome:

Patient did not respond to treatment. Patient died and four months later his brother was diagnosed with the same disease.

Case Study D:

History: A retired seventy-year-old male while sailing around the world fell on his yacht.

When examined by a local hospital (in Brazil) a fractured hip and pneumonia were found. He returned to the U.S. for a follow up.

Lab results were:

WBC 5.7

RBC 2.83

HGB 9.0

HCT 27.0

MCV 95.5

MCH 31.9

MCHC 33.4

RDW 23.4

PLT 101

Peripheral Blood Smear:

• 28% plasma cells
• Rouleaux was noted

Question 1: What do the above lab results indicate?

Additional testing

Protein Electrophoresis:

• Albumin 30.5%
• Alpha 1 2.6%
• Alpha 2 8.4%
• Beta 5.9%
• Gamma 53.6%

Question 2: What results are abnormal in the protein electrophoresis testing?

Additional testing

Immunoglobulin Quantitation:

              Result                Reference

IgG          307             723-1,685 mg/dl

IgA         1069                69-382 mg/dl

IgM           31                 63-277 mg/dl

Question 3: What is the final diagnosis based on ALL of the lab results?

Case Study E:

History: A 53-year-old male developed flu like symptoms with a high fever that progressed over the course of the week. Other symptoms include a weakness in his arms and legs, joint pains, painful headache and a sensitive scalp.

Lab results were:

WBC 3.7

RBC 3.45

HGB 11.0

HCT 34.0

MCV 89.5

MCH 29.7

MCHC 33.2

RDW 14.1

PLT 118

Differential:

• Segs 72%
• Lymphs 9%
• Mono 19%

Retic 2.0%

*MT noticed erythrocyte intracellular ring-like structures.

Chemistry:

• AST 4863
• ALT 1855
• ALP 72
• Total Bilirubin 0.6
• Direct Bilirubin 0.3
• Total protein 6.0

Additional Information: Several of the rings like structures were extracellular and the patient has never been outside of the U.S.

Question 1: Based on all of the results and information, what is the best possible diagnosis?

And how was the patient most likely infected with this disease?