Invented by Dr John Adler, a neurosurgeon at Stanford University, the Cyberknife is essentially a “surgical” technique that utilizes high doses of radiation delivered by sophisticated equipment to destroy tissue that is located in areas that are difficult to operate by open surgery. It is still an evolving technique, and clinical experience is limited.
This equipment has found many clinical applications, including cancer treatment. It is an adjunct to traditional cancer treatment in selected cases. However, it has limitations, and may not be useful for all patients, especially those with widespread metastatic disease. It has never been proven to extend overall survival.
It is already available in Apollo Hospital Chennai and HCG Bangalore (Bangalore Institute of Oncology), and expected to become shortly available in some other hospitals as well.
A comprehensive review on the clinical features, pathological basis, and treatment of polycythemia vera associated aquagenic pruritus was published in the September 2010 issue of the European Journal of Clinical Investigation.
Dr Ayalew Tefferi, the world renowned expert on myeloproliferative neoplasms, is the senior author of this paper. Dr Kamal S Saini, (medical oncologist, Institute Jules Bordet, Brussels) and Dr Mrinal M Patnaik (hematologist, Mayo Clinic, Rochester) are the other authors.
Should women who have had their uterus removed undergo regular Pap smear?
This issue has been addressed by Debbie Saslow et al in their landmark 2002 article “American Cancer Society Guideline for the Early Detection of Cervical Neoplasia and Cancer”. ACS recommends that women who have had total hysterectomy (with complete removal of the cervix) for benign conditions need not have Pap smear tests. Women with subtotal hysterectomy (where a portion of the cervix was left behind) must continue to have regular cervical Pap smears. Women who had total hysterectomy because of CIN 2 or 3, or who had CIN 2 or 3 before hysterectomy must undergo vaginal cuff cytology with Pap smear.
Q: The JAK2V617F mutation is classically seen in which hematological condition?
A: Polycythemia Vera (PV).
In 2005, this JAK2 gain-of-function mutation was detected in patients with PV, essential thrombocythemia, and primary myelofibrosis.
The 2008 WHO criteria for diagnosis of PV is as follows:
Hgb 418.5 g/100 ml in men or 16.5 g/100 ml in women or
Hgb or Hct 499th percentile of reference range for age/sex/altitude or
RCM 425% above mean normal predicted value, or Hgb 417 g/100 ml in
men or 15 g/100 ml in women if associated with 2 g/100 ml increase from
baseline that is not attributed to correction of IDA
Presence of the JAK2V617F or other functionally
similar mutations, such as the JAK2 exon 12 mutation
BM showing panmyelosis with prominent erythroid, granulocytic and
Serum Epo level below the reference range for normal
Endogenous erythroid colony formation in vitro
Reference: MM Patnaik and A Tefferi. The complete evaluation of erythrocytosis: congenital and acquired. Leukemia 2009.
Q: What is ‘Heriditary Xerocytosis‘?
A: A rare type of autosomal dominant (? chromosome 16) hemolytic anemia, heriditary xerocytosis or dessicocytosis is caused by potassium loss from the RBC unaccompanied by a proportional gain of sodium. Clinically manifests as mild hemolysis, high MCHC, slightly high MCV, stomatocytes, target cells, and spiculated cells. Splenectomy is not useful in this hemolytic anemia.
A patient recently sought my opinion during a cancer detection camp held at Kolkata. Here is her history and investigation reports.
A 31 year old lady, married, one child, resident of Bangladesh, came with a 11 month history of fever, chills, weight loss, joint pains (small joints and large joints, bilateral) and night sweats.
Investigations showed increased LDH, CRP (194), ESR, Ferritin (>1500), and low Hb (6.5).
Workup negative for HIV, malaria, and TB.
Blood cultures incl BACTEC negative thrice.
Immunology workup: Neg for ANA, dsDNA, Ro, La, RA factor.
Lymphoma workup negative.
ACE inhibitor slightly elevated 88.3 (N 8 – 52)
FNAC Rt submandibular gland : Normal salivary gland
Left axillary LN Bx: Follicular and paracortical T zone hyperplasia. CD45+, Neg for CD20, CD3, CD15. The
morphologically normal T cells were positive for Bcl2.
SGOT 169, SGPT 285 other liver functions WNL, RFT WNL
Coombs Direct positive, indirect neg
BM aspirate Neg for malignancy
CT scan shows multiple abdominal lymph nodes.
Patient is on steriods (Prednisolone 40 to 90 mg per day during different times) for the past 6 months, but intermittent fever persists.
On examination, she had pallor, tachycardia, moist skin, no significant peripheral lymphadenopathy, and her systemic examination was normal.
How would you have proceeded with this patient?